The 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.