This paper aimed to report a series of 19 cases of uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis. This retrospective descriptive observational study included the medical records of 19 patients seen at the Endocrinology Gynecology Sector of the Obstetrics and Gynecology Department of the Federal University of Paraná, and focused on clinical data, complementary exams, patient management, and follow-up. From the 229 patients with genital anomalies seen between 1984 and 2009, 19 (8.3%) were diagnosed with uterus didelphys with vaginal septum and renal agenesis. The patients had a median age of ±16.3 years. Eight subjects (42.6 %) reported abdominal pain; two of them (0.1%) had pelvic masses and seven (36.8%) had dysmenorrhea. Blood retention was confirmed by ultrasound and/ or magnetic resonance imaging of the pelvis, which revealed the existence of a duplicated uterus of the didelphys type with a cystic mass containing old blood obliterating the hemivagina. Urinary tract examination revealed the existence of ipsilateral renal agenesis. Unusual manifestations such as associated infection and rupture of the vaginal septum during sexual intercourse were also reported. The prevalence of uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis was 8.3% in a series of 229 genital anomalies. The observance of clinical features is essential for the early identification of the syndrome and the adequate management of the patients.