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The triad of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare entity. These cases are difficult to diagnose due to heterogeneous presentation and rarity of the condition. Ultrasonography and magnetic resonance imaging are important for initial diagnosis; however, laparoscopy is required for the confirmation of the diagnosis. Early and accurate diagnosis of this condition followed by prompt surgical therapy is essential to prevent complications and to preserve future fertility. Here we report a case of uterus didelphys with hemivagina and ipsilateral renal agenesis with hematometra and hematosalpinx in a young girl.

journal of south asian federation of obstetrics and gynaecology

Management of Hematometra in Single Horn of Didelphic Uterus with Obstructed Hemivagina and Ipsilateral Renal Agenesis Syndrome: A Case Report with Brief Review of Literature