Herlyn-Werner-Wunderlich syndrome (HWWs) is a rare congenital malformation that involves uterus dideslphys, obstructed hemivagina and ipsilateral renal agenesis. The obstructed vagina affects menstrual flow and causes related clinical features after menarche. Pelvic endometriosis is one of the common complications of HWWs. Resection of the vaginal septum can release the obstruction and result in good outcomes. However, in the case of cervical atresia, a rare variant of HWWs, ipsilateral hysterectomy is recommended because it is difficult to canalize cervical atresia surgically. Here we present a case of HWWs with cervical atresia complicated with pelvic endometriosis. She was treated with laparoscopic ovarian cystectomy followed by hormonal therapy. Six months after surgery, there is no evidence of recurrence of endometrioma.
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