Abstract
INTRODUCTION: Herlyn-Werner-Wunderlich Syndrome (HWWs) is a rare congenital anomaly. This abnormality has 5% percentage of the total Mullerian dysgenesis. The symptomatic patients come with varies condition, such as urinary incontinence, urinary retention endometriosis, pelvic infection and acute pelvic pain. Here we present investigation and management of HWWs with pelvic pain in adult woman. PRESENTATION OF CASE: A 23 years old woman came with pelvic pain. We found abnormalities of HWW syndrome i.e. uterus didelphys with obstructed hemivagina and right renal agenesis through ultrasound imaging, CT scan and MRI. After concluding the diagnosis, the patient underwent vaginal septum excision and vaginoplasty procedures to relieve obstruction which was the cause of pelvic pain. The symptom was improved after surgery and she felt no more pain after a year of follow up. DISCUSSION: The main symptom of HWWs is dysmenorrhea associated with obstruction mass or endometriosis. MRI with capability of tissue differentiation is the gold standard for diagnostic. The primary purpose of surgery is to release the obstruction and to prevent further complication. CONCLUSION: Rarely, HWWs causes an acute pelvic pain with secondary cystic mass finding. Surgery aimed to release obstruction will improve this symptom.