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

•OHVIRA syndrome presents at menarche with pain due to vaginal obstruction.•There are common pathways of renal, reproductive and anorectal embryology.•Pain is relieved by external vaginoplasty to drain the obstructed second vagina.•The obstructed uterus can carry pregnancies and should be preserved.

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.