We present a previously unreported combination of müllerian and wolffian anomalies of a septate uterus with double cervices, unilaterally obstructed vaginal septum, and ipsilateral renal agenesis; this constellation of findings may offer clues that could modify classic embryologic explanations. In spite of the young age of our patient (15-years old), a chief complaint of malodorous vaginal discharge, and absence of dysmenorrhea or any other symptoms of endometriosis, laparoscopic examination revealed severe endometriosis with dense adhesions, probably as a result of abundant menstrual regurgitation. Laparoscopic resection of endometriotic lesions, adhesiolysis, and vaginoscopic septotomy were successfully performed while preserving hymenal integrity.