It is generally treated surgically, with a hymenotomy or other surgery to remove any tissue that blocks the menstrual flow.

A number of twin gestations have occurred where each uterus carried its pregnancy separately. A recent example occurred on February 26, 2009, when Sarah Reinfelder of Sault Ste. Marie, Michigan delivered two healthy, although seven weeks premature, infants by cesarean section at Marquette General Hospital. It is possible that the deliveries occur at different times, thus the delivery interval could be days or even weeks.

In the United States, uterus didelphys is reported to occur in 0.1–0.5% of women. It is difficult to know the exact occurrence of this anomaly, as it may go undetected in the absence of medical and reproductive complications.

A simple cruciate incision followed by excision of tags of hymen allows drainage of the retained menstrual blood. A thicker transverse vaginal septum can be treated with Z-plasty. A blind vagina will require a partial or complete vaginoplasty. Hematosalpinx may require laprotomy or laparoscopy for removal and reconstruction of affected tube.

Infertility may require assisted reproductive techniques.

The first line of therapy after diagnosis typically involves the administration of the combined oral contraceptive pill, medroxyprogesterone acetate or a gonadotropin-releasing hormone agonist to suppress menstruation and thereby relieve pain. Surgically, cervical agenesis has historically been treated through hysterectomy (removal of the uterus) to relieve symptoms caused by hematocolpos (the accumulation of menstrual fluid in the vagina). Other surgical methods of management involve the creation of an anastomotic connection between the uterus and vagina by neovaginoplasty or recanalization of the cervix. Outcomes in these cases are generally poor, since the natural functions of the cervix—such as mucus production and providing a barrier against ascending infection—cannot be replicated. Furthermore, the success rate of uterovaginal anastomosis is less than 50% and most patients require multiple surgeries while many develop cervical stenotis. Despite this, several pregnancies have been reported in women with cervical agenesis who underwent surgical treatment.

Hematocolpos is a medical condition in which the vagina fills with menstrual blood. It is often caused by the combination of menstruation with an imperforate hymen. It is sometimes seen in Robinow syndrome, uterus didelphys, or other congenital conditions.

A related disorder is hematometra, where the uterus fills with menstrual blood. It presents after puberty as primary amenorrhoea, recurrent pelvic pain with a pelvic mass. This can be caused by a congenital stenosis of the cervix, or by a complication of a surgical treatment.

Cervical agenesis is estimated to occur in 1 in 80,000 females. It is often associated with deformity of the vagina; one study found that 48% of patients with cervical agenesis had a normal, functional vagina, while the rest of the cases were accompanied by vaginal hypoplasia.

Cryptomenorrhea or cryptomenorrhoea, also known as hematocolpos, is a condition where menstruation occurs but is not visible due to an obstruction of the outflow tract. Specifically the endometrium is shed, but a congenital obstruction such as a vaginal septum or on part of the hymen retains the menstrual flow. A patient with cryptomenorrhea will appear to have amenorrhea but will experience cyclic menstrual pain. The condition is surgically correctable.

The patient usually presents at the age of puberty when the commencement of menstruation blood gets collected in the vagina and gives rise to symptoms.

Before surgical intervention in adolescents, symptoms can be relieved by the combined oral contraceptive pill taken continuously to suppress the menstrual cycle or NSAIDs to relieve pain. Surgical treatment of the imperforate hymen by hymenotomy typically involves making cruciate incisions in the hymen, excising segments of hymen from their bases, and draining the vaginal canal and uterus. For affected girls who wish (or whose parents wish) to have their hymens preserved, surgical techniques to excise of a central flange of the hymen can be used. The timing of surgical hymen repair is controversial: some doctors believe it is best to intervene immediately after the neonatal period, while others believe that surgical repair should be delayed until puberty, when estrogenization is complete.

Imperforate hymen is the most common vaginal obstruction of congenital origin. Estimates of the frequency of imperforate hymen vary from 1 in 1000 to 1 in 10,000 females.

Renal agenesis is a medical condition in which one (unilateral) or both (bilateral) fetal kidneys fail to develop.

Unilateral and Bilateral Renal Agenesis in humans, mice and zebra fish has been linked to mutations in the gene GREB1L. It has also been associated with mutations in the genes "RET" or "UPK3A". in humans (see Rosenblum et al 2017 for review) and mice respectively.

Bilateral renal agenesis is a condition in which both kidneys of a fetus fail to develop during gestation. It is one causative agent of Potter sequence. This absence of kidneys causes oligohydramnios, a deficiency of amniotic fluid in a pregnant woman, which can place extra pressure on the developing baby and cause further malformations. The condition is frequently, but not always the result of a genetic disorder, and is more common in infants born to one or more parents with a malformed or absent kidney.