English

Abstract

Vaginal agenesis combined with a functional uterus is a rare type of Mullerian duct anomaly. Only 7-8% of patients with vaginal agenesis have a functional uterus. Women born with vaginal agenesis, cervical atresia combined with a functioning endometrium typically present with hematometra, disabling pelvic pain and progressively worsening endometriosis. Almost all need an abdominal hysterectomy for relief. We report a case of severe endometriosis in a 29 years old woman having a bicornuate uterus with cervical atresia and vaginal agenesis.