• Guifeng Jia, Wei Chai, Miao Cui, Yan Wen, Lifeng Cui, and Fengyan Gong.
• Department of Gynecology and Obsterics, The First Hospital of Jilin University, Changchun, Jilin, China Eastgene Laboratory, Flushing, New York.
• Medicine (Baltimore). 2018 Sep 1; 97 (36): e12004e12004.

IntroductionHerlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital abnormality of the urogenital tract characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It is usually diagnosed after menarche, with a clinical presentation of dysmenorrhea, recurrent abdominal pain, and irregular menses. However, it is rare to diagnose it during pregnancy, subsequently resulting in spontaneous abortion.Case PresentationA 22-year-old Chinese woman with HWWS whose left uterine pregnancy underwent spontaneous abortion presented with a right perforated obstructed hemivagina and right renal agenesis. The right vaginal septum was resected and the hematocolpos was drained, thereby relieving lower abdominal pain and preserving future fertility.ConclusionCo-presentation of unilateral renal agenesis and uterus didelphys should encourage clinicians to rule out HWWS. Early diagnosis and subsequent treatment can avoid possible serious complications.

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