Congenital anomalies that result in vaginal obstruction often present with an abdominal mass in the newborn period. Knowledge of the embryological development of the genitourinary tract and its variations is needed to properly evaluate the neonatal patient and to initiate therapy. Because of a high incidence of associated anomalies, a complete urologic evaluation should be undertaken before a definitive surgical repair. The methods of treatment for the various causes of vaginal atresia have shown long-term success and have resulted in appropriate psychosocial and sexual maturation.