Adam, AhmedEbrahim, Zaeem I.Engelbrecht, Matthys J.Van Heerden, Izak J.Moshokoa, Evelyn M.2010-11-092010-11-092009Adam, A, Ebrahim, ZI, Engelbrecht, MJ, Van Heerden, IJ & Moshokoa, EM 2009, 'Mayer-Rokitansky-Küster-Hauser syndrome associated with a urogenital sinus anomaly in a 4-year-old : report of a case', Current Urology, vol. 3, no. 2, pp. 103-105. [http://content.karger.com/ProdukteDB/produkte.asp?Aktion=JournalHome&ProduktNr=231997]1661-764910.1159/000189693http://hdl.handle.net/2263/15230The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare entity characterized by congenital aplasia of the vagina and uterus in the presence of normal ovarian function, in conjunction with a 46 XX karyotype. This condition is mostly signalled by primary amenorrhoea around the season of puberty. We report on its diagnosis in a 4-year-old child presenting with urinary incontinence. Also of interest in our index patient is the presence of a pure urogenital sinus anomaly. This extremely rare association has not been previously diagnosed in childhood.enKargerMayer-Rokitansky-Küster-Hauser syndromeUrogenital sinus anomalyPostprint Article