Scimitar syndrome is a rare anomaly, most commonly including partial pulmonary venous drainage into the inferior vena cava, hypoplasia of the right lung, and systemic collaterals from the aorta. We report a pediatric case in whom this rare syndrome was associated with intracardiac lesions and a large aberrant systemic arterial supply to the right lung that originated from the abdominal aorta and fistulated in the inferior vena cava.