Herlyn-Werner-Wunderlich (HWW) syndrome is a rare developmental anomaly that is consists of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. This rare entity is the spectrum of Mullerian duct anomalies (MDA) accompanied by developmental anomaly of one of Wolffian ducts. The present report demonstrated HWW syndrome and reviewed literatures in term of embryological etiology, clinical manifestation, radiographic findings and surgical management. In this case report is a 11-year-old girl presented with chronic pelvic pain. She had menarche at the age of 10 and her menstrual cycles were regular with moderate dysmenorrhea. Physical examination revealed palpable pelvic mass with tenderness. Transabdominal ultrasonography (US) and Magnetic resonance imaging (MRI) demonstrated uterine didelphys with right-sided hematometrocolpos and absent right kidney. Right hematosalpinx was also detected due to distal tubal occlusion from adhesion. These preoperative images can verify all of the features of this syndrome and correctly anticipated diagnosis was achieved. The patient underwent laparoscopic right tubal drainage with lysis of pelvic adhesion and hysteroscopic resection of vaginal septum. Her symptoms were improved uneventfully. In conclusion, HWW syndrome exhibits unique clinical presentation with characteristic radiographic findings and symptom can be relieved dramatically after receiving appropriate surgical management.

Keywords: Herlyn-Werner-Wunderlich syndrome, Uterine didelphys, hemivagina, MR findings and Ultrasonographic findings