Objective: To present a case of a unicornuate uterus with ipsilateral ovarian and renal agenesis. Design: Case report. Setting: Baskent University Faculty of Medicine, Department of Obstetrics and Gynecology, Adana, Turkey. Patient(s): A 48- year- old gravida 3 (para 3 with term deliveries) woman with a 6- month history of menometrorrhagia was admitted to our clinic. Our diagnosis was a pedunculated submucous leiomyoma that protruded into the vagina. Intervention(s): The patient chose to have a total abdominal hysterectomy and unilateral salpingoopherectomy. Main Outcome Measure(s): During laparotomy, a unicornuate uterus with a noncommunicating horn, together with ipsilateral ovarian agenesis, was observed. A total abdominal hysterectomy and unilateral salpingoopherectomy were performed successfully. Because we could not detect the left ovary and left ureter during the operation, we planned an abdominal ultrasonography and intravenous pyelography (IVP) postoperatively to demonstrate possible urinary tract abnormalities. Result(s): We detected left renal agenesis by IVP. Conclusion(s): We presented a very rare clinical condition that demonstrates a unicornuate uterus with a noncommunicating horn, and ipsilateral ovarian and renal agenesis concomitantly. The absence of one ovary and one kidney in our case may be explained by the abnormal development of organs derived from a unilateral urogenital ridge. Objective: To present a case of a unicornuate uterus with ipsilateral ovarian and renal agenesis. Design: Case report. Settings: Baskent University Faculty of Medicine, Department of Obstetrics and Gynecology, Adana, Turkey. Patient (s): A 48- old diagnosis of a pedunculated submucous leiomyoma that protruded into the vagina. Intervention (s): The patient chose to have a total Abdominal hysterectomy and unilateral salpingoopherectomy. Main Outcome Measure (s): During laparotomy, a unicornuate uterus with a noncommunicating horn, together with ipsilateral ovarian agenesis, was observed. A total abdominal hysterectomy and unilateral salpingoopherectomy were performed successfully. Because we could not detect the left ovary and left ureter during the operation, we planned an abdominal ultrasonography and intravenous pyelography (IVP) postoperatively to demonstrate p Conclusion (s): We presented a very rare clinical condition that demonstrates a unicornuate uterus with a noncommunicating horn, and ipsilateral ovarian and renal agenesis concomitantly. The absence of one ovary and one kidney in our case may be explained by the abnormal development of organs derived from a unilateral urogenital ridge.