We report a case of a single system solitary ectopic ureter to the rectum with bladder agenesis in a viable newborn male. Only 2 cases of ectopic ureter to the rectum and 18 cases of bladder agenesis have been reported in viable newborns. To our knowledge, this case constitutes the first reported combination of these defects in a living neonate. Associated anomalies also included renal dysplasia, anal stenosis, and urethral-rectal fistula. Nephrectomy is often necessary to prevent infection or hypertension.