Congenital atresia of the uterine cervix is a rare Müllerian anomaly. About half of the reported cases are associated with pelvic endometriosis. We describe a 20-year-old woman with congenital cervical atresia and pelvic endometriosis whose manifesting symptoms were primary amenorrhea and pelvic pain. Surgical treatment consisted of excision of bilateral ovarian endometrial cysts and hysterectomy. A review of the literature suggests that successful surgical management with regard to preservation of reproductive potential may be possible in some cases, but treatment must be individualized and hysterectomy is sometimes indicated.