Background: We report a case of monochorionic, monoamniotic female twins with discordant congenital reproductive tract anomalies.
Case: We present the case of a 17-year-old monochorionic-monoamniotic female twin with Mayer-von Rokitansky-Küster-Hauser Syndrome (MRKH) and her twin sister, who had with normal female reproductive system. Monozygosity is investigated by the complete history, physical, and imaging data, antenatal and birth reports of both the patient and her monozygotic twin sister. Examination of these documents proves monozygosity of the sisters, and imaging studies demonstrate MRKH in one twin and normal female genitalia in the other.
Summary and conclusion: This case is presented as proof of MRKH discordance in monozygotic twins.