A 20-year-old woman had urinary incontinence since childhood. She self-managed her symptoms by using incontinence pads and she admitted never having been to a urologist. When she consulted a urologist for cystitis, ultrasonography could not locate the presence of a right kidney. She was suspected of having a contracted kidney and was referred to our hospital for further examinations and treatment. An enhanced computed tomography scan showed a contracted right kidney, which was located on the surface of the inferior vena cava. Magnetic resonance imaging showed that the right ureter extended into the vagina. Cystoscopy showed the absence of a right ureteral orifice, and an ectopic orifice was identified in the vagina. Laparoscopic nephroureterectomy was performed based on the diagnosis of a hypoplastic kidney with an ectopic ureter. We removed the ureter as far as the vagina because a residual ureteral segment could cause infection. Postoperatively, the patient had no complications, and her uterine artery was successfully saved. The patient achieved complete continence after the operation.