We present a case of a patient with Uterus bicornis bicollis, imperforate hemivagina and ipsilateral renal agenesis. This group of congenital malformations is often asymptomatic until puberty, when it presents as cyclic dysmenorrhoea, leucorrhoea or a pelvic mass. Magnetic resonance imaging is becoming the preferred modality for delineation of uterine malformations. When congenital abnormalities of the reproductive tract are encountered, a search should also be made for renal anomalies. Patients with Uterus bicornis bicollis and unilateral imperforate vagina are often seen with pain severe enough to mimic an acute abdomen. It is important to keep this unusual congenital malformation in mind in the differential diagnosis of vaginal discharge, pelvic mass and/or abdominopelvic pain in young women so as to avoid inappropriate surgical procedures.