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Didelphic uterus with obstructed hemivagina and ipsilateral renal agenesis is a rare condition. It usually presents with pelvic pain following the menarche, dysmenorrhoea, and an increase in abdominal volume or a palpable mass due to unilateral haematocolpos. We present the case of a 13-year-old girl who referred with recurrent pelvic pain, mainly at the time of menses, and irregular menstrual cycle complaints in this report. The patient underwent ultrasonography and magnetic resonance (MR) imaging of the pelvis was performed. The diagnosis was uterus didelphys with obstructed hemivagina and ipsilateral renal agenesia. Laparotomy was performed for diagnosis and treatment purposes. Two separated hemiuteri and two cervices with hematometra and hematocolpos on the right side and ipsilateral renal agenesis were detected. The vaginal septum was excised completely and Strassman metroplasty was performed. Her complaints were resolved and she was absolutely asymptomatic after surgery. Diagnosis and management of this congenital anomaly is challenging due to the complexity of the anatomic structures, nonspecific complaints, and heterogenic presentation. These anomalies must always be considered while working-up female patients presenting with episodic abdominal pain and abdominopelvic mass.

Uterus didelphys with an obstructed unilateral vagina and ipsilateral renal agenesis: A rare cause of dysmenorrhoea