We report on an 18-year-old woman with a combined urogynecological malformation who presented with anuria. At the age of 8 years she presented elsewhere with urometrocolpos secondary to a left hypoplastic kidney and an ectopic ureter that inserted into the vagina. A retroiliac right ureter also was identified. She underwent surgical treatment and was reported in the urological literature. At menarche she began to have severe dysmenorrhea. After multiple hospitalizations elsewhere without diagnosis she presented to our emergency room with anuria. The anuria was the direct result of additional, previously unidentified malformations, consisting of uterus duplex bicollis and vaginal hypoplasia. We emphasize the importance of identifying all components of the genital and urological systems whenever anomalies are present in either system.