We report the case of 23 year-old woman with OHVIRA syndrome (obstructed hemivagina and ipisilateral renal anomaly) discovered during management for right renal failure. Non-specific symptoms such as lower abdominal pain, dysmenorrhea, and genital bleeding sometimes occur with congenital uterine anomalies such as this. It is very difficult to diagnose OHVIRA syndrome accurately without ultrasound and magnetic resonance imaging, and patients can develop severe complications as a result of delays in diagnosis: endometriosis, pelvic adhesions, or infertility can occur through backflow of genital bleeding because of vaginal septum. In our patient we managed to avoid severe complications by surgically resecting the vaginal septum. She was treated within an appropriate time frame and without complications. Fortunately, after the surgery she managed to become pregnant in the left side of the uterus.

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