Abstract
INTRODUCTION: Herlyn-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 PRESENTATION: A 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. CONCLUSION: Co-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.