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.