The congenital melanocytic nevus appears as a circumscribed, light brown to black patch or plaque, potentially very heterogeneous in consistency, covering any size surface area and any part of the body.

As compared with a melanocytic nevus, congenital melanocytic nevi are usually larger in diameter and may have excess terminal hair, a condition called hypertrichosis. If over 40 cm projected adult diameter with hypertrichosis, it is sometimes called giant hairy nevus; more usually these largest forms are known as large or giant congenital melanocytic nevus. The estimated prevalence for the largest forms is 0.002% of births.

Melanocytic Nevi often grow proportionally to the body size as the child matures. As they mature, they often develop thickness, and become elevated, although these features can also be present from birth. Prominent terminal hairs often form, especially after puberty. With maturity, the nevus can have variation in color, and the surface might be textured with proliferative growths.

Neurocutaneous melanosis is associated with the presence of either giant congenital melanocytic nevi or non-giant nevi of the skin. It is estimated that neurocutaneous melanosis is present in 2% to 45% of patients with giant congenital melanocytic nevi. Neurocutaneous melanosis is characterized by the presence of congenital melanocytic nevi on the skin and melanocytic tumors in the leptomeninges of the central nervous system.

Hypermelanotic nevi must be differentiated from other types of pigmented skin lesions, including:

- Lentigo simplex

- Solar lentigo

- Café au lait macule

- Ink-spot lentigo

- Mucosal melanotic macule

- Mongolian spot (dermal melanocytosis)

Additional types of nevi do not involve disorders of pigmentation or melanocytes. These additional nevi represent hamartomatous proliferations of the epithelium, connective tissue, and vascular malformations.

Congenital melanocytic nevi may be divided into the following types:

- "Small-sized congenital melanocytic nevus" is defined as having a diameter less than 2 cm.

- "Medium-sized congenital melanocytic nevus" is defined as having a diameter more than 2 cm but less than 20 cm.

- "Giant congenital melanocytic nevus" (also known as "Bathing trunk nevus," "Garment nevus," "Giant hairy nevus", and "Nevus pigmentosus et pilosus") is defined by one or more large, darkly pigmented and sometimes hairy patches.

Phakomatosis pigmentovascularis is a rare neurocutanous condition where there is coexistence of a capillary malformation (port-wine stain) with various melanocytic lesions, including dermal melanocytosis (Mongolian spots), nevus spilus, and nevus of Ota.

Nevoid melanoma is a cutaneous condition that may resemble a Spitz nevus or an acquired or congenital melanocytic nevus.

Blue nevi may be divided into the following types:

- A "patch blue nevus" (also known as an "acquired dermal melanocytosis", and "dermal melanocyte hamartoma") is a cutaneous condition characterized by a diffusely gray-blue area that may have superimposed darker macules.

- A "blue nevus of Jadassohn–Tièche" (also known as a "common blue nevus", and "nevus ceruleus") is a cutaneous condition characterized by a steel-blue papule or nodule.

- A "cellular blue nevus" is a cutaneous condition characterized by large, firm, blue or blue-black nodules.

- An "epithelioid blue nevus" is a cutaneous condition most commonly seen in patients with the Carney complex.

- A "deep penetrating nevus" is a type of benign melanocytic skin tumor characterized, as its name suggests, by penetration into the deep dermis and/or subcutis. Smudged chromatic is a typical finding. In some cases mitotic figures or atypical melanocytic cytology are seen, potentially mimicking a malignant melanoma. Evaluation by an expert skin pathologist is advisable in some cases to help differentiate from invasive melanoma.

- An "amelanotic blue nevus" (also known as a "hypomelanotic blue nevus") is a cutaneous condition characterized by mild atypia and pleomorphism.

- A "malignant blue nevus" is a cutaneous condition characterized by a sheet-like growth pattern, mitoses, necrosis, and cellular atypia.

Phakomatosis pigmentovascularis is subdivided into five types:

- Type 1 PWS + epidermal nevus

- Type 2 (most common): PWS + dermal melanocytosis +/- nevus anemicus

- Type 3: PWS + nevus spilus +/- nevus anemicus

- Type 4: PWS + nevus spilus + dermal melanocytosis +/- nevus anemicus

- Type 5: CMTC (Cutis marmorata telangiectatica congenita) + dermal melanocytosis

They all can contain capillary malformation. Type 2 is the most common and can be associated with granular cell tumor. Some further subdivide each type into categories A & B; with A representing oculocutaneous involvement and subtype B representing extra oculocutaneous involvement. Others have proposed fewer subtypes but currently this rare entity is mostly taught as having five subtypes currently.

Nevus sebaceus or sebaceous nevus (the first term is its Latin name, the second term is its name in English; also known as an "organoid nevus" and "nevus sebaceus of Jadassohn") is a congenital, hairless plaque that typically occurs on the face or scalp. Such nevi are present at birth, or early childhood, affecting males and females of all races equally. The condition is named for an overgrowth of sebaceous glands in the area of the nevus.

Skin growths such as benign tumors and basal cell carcinoma can arise in sebaceous nevi, usually in adulthood. Rarely, sebaceous nevi can give rise to sebaceous carcinoma. However, the rate of such malignancies is now known to be less than had been estimated. For this reason, excision is no longer automatically recommended.

Mucinous nevus (also known as "Nevus mucinosus") is a rare cutaneous condition characterized by hamartoma that can be congenital or acquired.

Blue nevus (also known as "blue neuronevus", "dermal melanocytoma", and "nevus bleu") is a type of melanocytic nevus. The blue colour is caused by the pigment being deeper in the skin than in ordinary nevi. In principle they are harmless but they can sometimes be mimicked by malignant lesions, i.e. some melanomas can look like a blue nevus.

A "hemangioma of infancy", colloquially called a strawberry mark, is a benign self-involuting tumor (swelling or growth) of endothelial cells, the cells that line blood vessels. It usually appears during the first weeks of life and resolves by age 10. It is the most common tumor of infancy.

PHACES Syndrome, a rare condition that often involves brain, heart, and arterial abnormalities, is generally accompanied by the presence of large facial hemangiomas. In such cases, what appears to be a small bruise or birthmark may grow rapidly and take on a puffy appearance in the first days or weeks of life.

Port-wine stains, also known as nevus flammeus and sometimes mistaken for strawberry marks, are present at birth and range from a pale pink in color, to a deep wine-red. Irregular in appearance, they are usually quite large, and caused by a deficiency or absence in the nerve supply to blood vessels. This causes vasodilation, the dilation of blood vessels, causing blood to pool or collect in the affected area. Over time, port-wine stains may become thick or develop small ridges or bumps, and do not fade with age. Such birthmarks may have emotional or social repercussions. Port-wine stains occur in 0.3% of the population, equally among males and females. They frequently express unilaterally, i.e., on only one side, not crossing the midline of the body. Often on the face, marks on the upper eyelid or forehead may be indicative of a condition called Sturge-Weber syndrome. Additionally, port-wine stains in these locations may be associated with glaucoma and seizures.

Some sources equate the term mole with "melanocytic nevus". Other sources reserve the term "mole" for other purposes such as the animal of the same name.

Melanocytic nevi represent a family of lesions. The most common variants are:

- Location:

- Junctional nevus: the nevus cells are located along the junction of the epidermis and the underlying dermis. A junctional nevus is flat and brown to black.

- Compound nevus: a mixture of junctional and intradermal proliferation. Compound nevi are slightly raised and brown to black. Beauty marks are usually compound nevi of either the acquired variety or congenital variety.

- Intradermal nevus: the nevus cells are located in the dermis only. Intradermal nevi are raised; most are flesh-colored (not pigmented).

- Dysplastic nevus (nevus of Clark): usually a compound nevus with cellular and architectural dysplasia. Like typical moles, dysplastic nevi can be flat or raised. While they vary in size, dysplastic nevi are typically larger than normal moles and tend to have irregular borders and irregular coloration. Hence, they resemble melanoma, appear worrisome, and are often removed to clarify the diagnosis. Dysplastic nevi are markers of risk when they are numerous (atypical mole syndrome). According to the National Cancer Institute (NIH), doctors believe that, when part of a series or syndrome of multiple moles, dysplastic nevi are more likely than ordinary moles to develop into the most virulent type of skin cancer called melanoma.

- Blue nevus: It is blue in color as its melanocytes are very deep in the skin. The nevus cells are spindle shaped and scattered in deep layers of the dermis. The covering epidermis is normal.

- Spitz nevus: a distinct variant of intradermal nevus, usually in a child. They are raised and reddish (non-pigmented). A pigmented variant, called the 'nevus of Reed', typically appears on the leg of young women.

- Acquired nevus: Any melanocytic nevus that is not a congenital nevus or not present at birth or near birth. This includes junctional, compound and intradermal nevus.

- Congenital nevus: Small to large nevus present at or near time of birth. Small ones have low potential for forming melanomas, however the risk increases with size, as in the giant pigmented nevus.

- Giant pigmented nevus: these large, pigmented, often hairy congenital nevi. They are important because melanoma may occasionally (10 to 15%) appear in them.

- Intramucosal nevus: junctional nevus of the mucosa of the mouth or genital areas. In the mouth, they are found most frequently on the hard palate.

- Nevus of Ito and nevus of Ota: congenital, flat brownish lesions on the face or shoulder.

- Mongolian spot: congenital large, deep, bluish discoloration which generally disappears by puberty. It is named for its association with East Asian ethnic groups but is not limited to them.

- Recurrent nevus: Any incompletely removed nevus with residual melanocytes left in the surgical wound. It creates a dilemma for the patient and physician, as these scars cannot be distinguished from a melanoma.

People with visible marks generally feel fine (physically) and can act normally, but when it is mentioned, they may become withdrawn and self-conscious. Some children may have low self-esteem due to the condition.

CMTC is an uncommon, sporadic congenital vascular malformation characterized by a generalized or localized reticulated cutaneous vascular network.

Cutaneous lesions described in patients with CMTC include nevus flammeus, hemangioma, nevus anemicus, café-au-lait spots, melanocytic nevus, aplasia cutis and acral cyanosis.

It has a marbled bluish to deep-purple appearance. The dark skin lesions often show a palpable loss of dermal substance. The reticulated mottling frequently appears more prominent in a cold environment (physiologic cutis marmorata), but tends not to disappear with warming. Hence, the erythema may be worsened by cooling, physical activity, or crying.

CMTC frequently involves the extremities, with the lower extremities involved most commonly, followed by the upper extremities, and then the trunk and face. The lower extremities often show atrophy and seldom show hypertrophy resulting in limb circumference discrepancy.

When located on the trunk, the lesions of CMTC tend to show mosaic distribution in streaks with a sharp midline demarcation seen across the abdomen. The lesions are primarily localized, but can be segmental or generalized, often unilateral in appearance. Diffuse involvement of the skin is usually not observed.

Although its course is variable, the majority of lesions in mild cases fade by adolescence. Ulceration and secondary infection are complications in severe cases and can be fatal if present in the neonatal period.

According to the American Academy of Dermatology, the most common types of moles are skin tags, raised moles and flat moles. Benign moles are usually brown, tan, pink or black (especially on dark-colored skin). They are circular or oval and are usually small (commonly between 1–3 mm), though some can be larger than the size of a typical pencil eraser (>5 mm). Some moles produce dark, coarse hair. Common mole hair removal procedures include plucking, cosmetic waxing, electrolysis, threading and cauterization.

Café au lait" spots or café au lait" macules are flat, pigmented birthmarks. The name "café au lait" is French for "coffee with milk" and refers to their light-brown color. They are also called "giraffe spots" or "coast of Maine spots".

They are caused by a collection of pigment-producing melanocytes in the epidermis of the skin.

These spots are typically permanent, and may grow or increase in number over time.

Café au lait spots are often harmless, but may be associated with syndromes such as neurofibromatosis type 1 and McCune–Albright syndrome.

Nevus flammeus may be divided as follows:

- Nevus flammeus nuchae

- Midline nevus flammeus

Melanoma with features of a Spitz nevus (also known as a "Spitzoid melanoma") is a cutaneous condition characterized histologically with tissue similar to a spitz nevus and with overall symmetry and a dermal nodule of epithelioid melanocytes that do not mature with progressively deeper dermal extension.

Nevus comedonicus (also known as a "comedo nevus") is characterized by closely arranged, grouped, often linear, slightly elevated papules that have at their center keratinous plugs resembling comedones.

Hair follicle nevus (also known as a "Vellus hamartoma") is a cutaneous condition that presents as a small papule from which fine hairs protrude evenly from the surface.

An Apocrine nevus is an extremely rare cutaneous condition that is composed of hyperplastic mature apocrine glands.

Phakomatosis pigmentokeratotica is a rare neurocutanous condition characterized by the combination of an organoid sebaceous nevus and speckled lentiginous nevus. It is an unusual variant of epidermal naevus syndrome. It was first described by Happle "et al". It is often associated with neurological or skeletal anomalies such as hemiatrophy, dysaesthesia and hyperhidrosis in a segmental pattern, mild mental retardation, seizures, deafness, ptosis and strabismus.

A capillary hemangioma (also known as an Infantile hemangioma, Strawberry hemangioma, and Strawberry nevus) is the most common variant of hemangioma which appears as a raised, red, lumpy area of flesh anywhere on the body, though 83% occur on the head or neck area. These marks occur in about 10% of all births, and usually appear between one and four weeks after birth. It may grow rapidly, before stopping and slowly fading. Some are gone by the age of 2, about 60% by 5 years, and 90–95% by 9 years. Capillary hemangioma is a vascular anomaly.

Capillary hemangiomas occur 5 times more often in female infants than in males, and mostly in Caucasian populations. Additionally, low birthweight infants have a 26% chance of developing a hemangioma.

It is the most common tumor of orbit and periorbital areas in childhood. It may occur in the skin, subcutaneous tissues and mucous membranes of oral cavities and lips as well as in the liver, spleen and kidneys. While this birthmark may be alarming in appearance, physicians generally counsel that it be left to disappear on its own, unless it is in the way of vision or blocking the nostrils.

Café au lait spots are usually present at birth, permanent, and may grow in size or increase in number over time.

Cafe au lait spots are themselves benign and do not cause any illness or problems. However, they may be associated with syndromes such as Neurofibromatosis Type 1 and McCune-Albright syndrome.

The size and shape of the spots do not have any meaning or implications with regards to diagnosis of associated syndromes.