Uterus didelphys with unilateral imperforate vagina and ipsilateral renal agenesis

Uterus didelphys with unilateral imperforate vagina is rare. Interestingly, in all reported cases in which the urinary tract was investigated renal agenesis was found on the side of the obstructed hemivagina. We report 2 cases of this unusual and interesting condition, and review the embryogenesis of müllerian duct abnormalities associated with renal agenesis, as well as the clinical findings, diagnosis and management. We stress the importance of prompt and accurate diagnosis, and treatment as a means to avoid unnecessary surgical procedures.