Ectopic ureter occurs most commonly in association with ureterocele or duplication of the ureter. It is caused by a delay in or failure of separation of the ureteric bud from the mesonephric duct during embryologic development. Vaginal agenesis is the second most common cause of primary infertility in women after gonadal dysgenesis. Agenesis of the vagina in karyotypic females may be accompanied by other defects of the urogenital system [1]. We describe a rare combination of Mayer–Rokitansky–Kuster–Hauser syndrome and ectopic ureter with urinary incontinence.