Herlyn-Werner-Wunderlich syndrome is a rare syndrome caused by a disturbance in the fusion of the Mullerian ducts characterized by uterus didelphys, unilateral obstructed hemivagina (hematocolpos) and ipsilateral renal anomalies. The syndrome is also known as OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly). The patients are usually diagnosed after menarche when they present with lower abdominal pain and occasionally a palpable mass in the pelvis.