Agenesis of the bladder is a rare congenital anomaly, recorded most often in stillborn infants. Renal agenesis and other severe malformations are frequently associated. An apparent failure of the infraureteral tissue of the mesonephric duct to develop into trigone and proximal urethra seems to be the cause of this anomaly. We report a case of bladder agenesis with anal atresia, left renal dysplasia, and bicornuate uterus.