Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) syndrome with imperforate anus

Abstract OHVIRA syndrome (Obstructed hemivagina with ipsilateral renal agenesis) is a rare Mullerian duct anomaly that results in uterine didelphys, obstructed vaginal vault, and unilateral renal agenesis. We report an 11 year old girl with a history of imperforate anus presenting with progressive abdominal pain. This represents the first documented case of OHVIRA syndrome in association with an ano-rectal malformation.