The MRKH-syndrome is a congenital Müllerian duct anomaly and characterized by agenesis of the uterus and upper part of the vagina with normal ovarian function and female karyotype. Possible associated malformations include renal dysplasia, skeletal anomalies, or hearing defects. Its etiology is still unclear, ranging from a monogenic origin to a multifactorial or nongenetic etiology. Uterus remnants are seen in at least 50% of all patients. Patients clinically present during adolescence with primary amenorrhea or refer to the impossibility of cohabitation. For initial diagnostics, transabdominal ultrasound can be performed to verify the absence of a uterus and presence of ovaries. Magnetic resonance imaging serves as the gold standard for the confirmation of uterovaginal agenesis. In the therapeutical management of vaginal agenesis, the nonsurgical and surgical techniques can be distinguished. Nonsurgical methods are based on a mechanical dilatation of the existing tissue. Surgical procedures comprise the use of autogenic transplants or the creation of a neovagina through dilatation with a traction device. Besides surrogacy, uterus transplantation has additionally now arisen as an infertility treatment to provide motherhood.