Herlyn Werner Wunderlich syndrome: A case of obstructed hemivagina with ipsilateral renal agenesis (OHVIRA syndrome) and didelphic uterus

We report a rare case of obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome with a didelphic uterus. A 22-year-old female presented with infertility for one year. Imaging studies revealed an absent left kidney, massive hematometrocolpos on the left side with normal other side. She was diagnosed with OHVIRA syndrome. She underwent septostomy of the vaginal septum, drainage of hematometrocolpos and hysterolaparoscopy that showed a bicornuate uterus and hematosalpinx. Given the rarity of the syndrome it is frequently misdiagnosed. Delayed diagnosis can lead to endometriosis.