Ureteral atresia is rare and usually associated with loss of renal function. Association with other urinary malformation remains exceptional. We report a case of distal ureteral atresia associated with a double excretory system, with preservation of renal function in a 3-year-old boy. Abdominal ultrasound showed voluminous cystic mass. CT-scan demonstrated a malrotated right kidney with double excretory system one of which was atretic. The child had a right partial superior ureter nephrectomy with removal of the atretic ureter. Outcome was good after 6 months follow-up.