Herlyn-Werner Wunderlich Syndrome (HWWS) is a rare urogenital tract malformation disease, characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal anomalies. It is also been named obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome or oblique vaginal septum syndrome (OVSS). This disease can be classified into four subtypes according to its anatomical features and the extent of menstrual obstruction. Patients will display various symptoms such as pelvic pain, abnormal vaginal discharge, or compression symptoms. The diagnosis of HWWS can be made by pelvic examination and proper imaging methods including ultrasound and MRI. Vaginal septum resection surgery is the most effective treatment method to relieve obstruction, prevent complications, and preserve fertility.