Background. Müllerian duct malformations delineate a miscellaneous group of congenital anomalies that result from arrested development, abnormal formation, or incomplete fusion of the mesonephric ducts. Case. This paper describes the diagnosis and management of a noncommunicating rudimentary horn complicated by severe pelvic pain and associated endometriosis. Conclusion. This condition was diagnosed by laparoscopy and hysteroscopy examination. Operative videolaparoscopy proved to be a successful approach for the treatment of this congenital Müllerian anomaly.