A case of a female subject with a unilateral vaginal ectopic ureter associated with a single system, ipsilateral hypoplastic pelvic kidney and bicornuate uterus is reported. Although there are many reports of concomitant urinary and reproductive system malformations, a review of the literature shows these unique associations in the same patient to be extremely rare. This malformation is not limited to any combination of abnormal development of the mesonephric(wolffian) and paramesonephric(mllerian) ducts in the development of the urogenital system. Embryological aspects as well as clinical presentation, diagndstic approach and treatment applicable to this case are discussed.