An unusual presentation of uterus didelphys with obstructed hemivagina with ipsilateral renal agenesis.

OBJECTIVE: To report a rare müllerian anomaly of uterus didelphys with obstructed hemivagina and renal agenesis, and its unusual presentation that mimicked an ischiorectal swelling.
DESIGN: Case report.
SETTING: Private clinic and surgical center. PATIENT(S): A 17-year-old woman referred for evaluation of a suspected müllerian anomaly. INTERVENTION(S): Clinical, hysteroscopic, and laparoscopic evaluation of the anomaly, with treatment and follow-up assessments. MAIN OUTCOME MEASURE(S): Assessment of the anomaly according to the standard müllerian classification system with a subsequent literature search. RESULT(S): Successful drainage of hematocolpos and hematometra, and subsequent normal menstruation. CONCLUSION(S): This rare müllerian anomaly, also known as Herlyn-Werner-Wunderlich syndrome (HWWS), presented as a huge paravaginal mass. Good long-term outcome occurred after vaginal septectomy. This diagnosis should be suspected in women who have a pelvic mass and ipsilateral renal agenesis.