Systemic artery-pulmonary vein fistula with a pulmonary left-left shunt is extremely rare. In this report, we present the case of a 3-month-old infant with enlarged right upper lobe systemic artery-pulmonary vein fistula and signs and symptoms of vascular tracheal compression and congestive heart failure. Two major aberrant arteries with separate origins came from the descending thoracic aorta and entered the right upper pulmonary lobe where they branched into many vascular channels and promptly drained into the enlarged right upper pulmonary vein. One of the two aberrant arteries was successfully coil embolized, and the other aberrant artery spontaneously closed after cardiac catheterization.