Tehran University of Medical Sciences Frontiers in Emergency Medicine 2717-3593 5 3 2021 03 04 e35 e35 Zahra Tavoli Department of Obstetrics and Gynecology, Ziaeian Hospital, Tehran University of Medical Science, Tehran Iran Ali Montazeri Population Health Research Group, Health Metrics Research Centre, Iranian Institute for Health Sciences Research, Tehran, Iran 2020 12 21 2020 12 29 Introduction: Uterus didelphys with obstructed hemivagina associated with ipsilateral renal agenesis (OHVIRA syndrome) is a rare female urogenital malformation and delay in its diagnosis could lead to several complications. Case presentation: A 21-year-old virgin woman was admitted to the emergency department (ED) with severe abdominal pain, without fever and vaginal discharge. She reported a history of cyclic abdominopelvic pain and dysmenorrhea for 5 years. The primary diagnosis (OHVIRA syndrome) was made using ultrasonography, spiral computed tomography (CT) and magnetic resonance imaging (MRI). In addition, laparoscopy was performed to confirm diagnosis and drain hematosalpinx. Then, hysteroscopy was carried out for septum resection and catheter insertion. At one-month follow-up the ultrasonography showed normal left hemicavity of uterus associated with significant decrease in dysmenorrhea. Conclusion: Being aware of OHVIRA syndrome and clinical suspicion of this rare anomaly are essential for making a timely diagnosis, preventing complications, relieving symptoms, and preserving future fertility. https://fem.tums.ac.ir/index.php/fem/article/view/615 https://fem.tums.ac.ir/index.php/fem/article/download/615/344