Background A 14-year-old girl with a solitary right kidney had continuous urinary incontinence. Four months previously she had undergone surgical resection of a vaginal septum associated with uterus didelphys, which was causing obstructed menstrual flow. She was toilet trained at the age of 2 years, had a normal voiding pattern, and had no history or family history of incontinence. Investigations Pelvic examination, abdominal and pelvic ultrasonography, renal scintigraphy, voiding cystourethrography, abdominal and pelvic MRI, fluoroscopic retrograde vaginography, vaginoscopy, cystourethroscopy after administration of indigo carmine, laparoscopy, and pathologic examination of the excised specimen. Diagnosis Ectopic ureter draining into the vagina associated with a contralateral dysplastic kidney. Management Laparoscopic nephrectomy of the left renal remnant and ligation of the left distal ureter.