Objective - to optimize the results of surgical correction and rehabilitation of patients with congenital uterovaginal anomalies in combination with endometriosis. Material and methods. 198 patients with congenital uterovaginal anomalies were operated from 2013 to 2015. All patients were divided into two groups: 105 patients with combined external genital endometriosis (group 1), 93 patients without endometriosis (group 2). A complete clinical and laboratory examination, ultrasound examination of the pelvic organs and kidney, magnetic resonance imaging were performed. Results. The reasons for the treatment of patients were pain in 17.2%, primary infertility in 41.5%, secondary infertility in 18.6% and recurrent miscarriage in 26.2% of cases. Surgical correction of uterovaginal anomalies, excision and coagulation of endometriotic lesions were made. During operative treatment concomitant gynecological pathology was diagnosed in 103 patients. The frequency of external genital endometriosis in patients with congenital uterovaginal anomalies was 53%. We also visualized endometriotic lesions on pelvic peritoneum in 20% of patients with aplasia of the uterus and vagina without functional rudimental horns. Conclusions. The laparoscopy revealed no significant differences in endometriosis rate among patients with and without outflow obstruction of menstrual blood. Retrograde menstruation followed by implantation of endometrial cells on the pelvic peritoneum was not the cause of development of external genital endometriosis in all women with uterovaginal malformations.