Herlyn-Werner-Wunderlich syndrome in a multiparous female.

Herlyn-Werner-Wunderlich syndrome is a rare complex congenital anomaly of the urogenital tract involving Mullerian ducts and mesonephric ducts. It is also called OHVIRA syndrome (Obstructed Hemivagina and Ipsilateral Renal agenesis). It is characterized by a triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Patients usually present after menarche with pelvic pain, dysmenorrhea, mass, and rarely with primary infertility in later years. We report a case of a multiparous female who presented to the hospital with pain in lower abdomen for the past 2 months and acute retention of urine for 1 day. This is an atypical presentation in a multiparous female that has been described in a single case report so far. Intravenous pyelogram and MRI of the patient revealed uterine didelphys, obstructed right hemivagina causing hematohemicolpos and right renal agenesis. Thorough knowledge of imaging features can enable a radiologist to make a correct diagnosis even in an atypical presentation.