Uterus Didelphys with Obstructed Hemivagina and Contralateral Multicystic Dysplastic Kidney

Müllerian anomalies are often associated with urinary tract anomalies. Herlyn-Werner-Wunderlich syndrome or obstructed hemivagina and ipsilateral renal anomaly are rare Müllerian anomaly comprising uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. We report an unusual case of uterus didelphys with obstructed hemivagina and contralateral (not ipsilateral) multicystic dysplastic kidney. An early diagnosis is critical to prevent complications such as adhesions with subsequent infertility. A careful evaluation of the female reproductive tract may be necessary in children with not only renal aplasia but also lateral renal anomalies such as multicystic dysplastic kidney.