Reported is the first surgically corrected case of tetralogy of Fallot with anomalous origin of the left pulmonary artery from the ascending aorta. The diagnosis of origin of the left pulmonary artery from the aorta depends on an aortogram, but clinical hints are increased vascularity in the left lung compared with the right lung and a continuous murmur. In tetralogy of Fallot, when there is late filling of the left pulmonary artery after a right ventricular cineangiogram, anomalous origin of the left pulmonary artery should be suspected and confirmed by an aortogram. Differentiation from complete absence of the left pulmonary artery, which is also often associated with tetralogy of Fallot, is possible by chest roentgenogram.