Tetralogy of Fallot with absent pulmonic valve is a rare cardiac malformation. The most significant symptoms during early infancy are secondary to bronchial compression resulting from the dilated pulmonary arteries. The clinical diagnosis was confirmed by echocardiography, cardiac catheterization and cardioangiography. A case of TOF with absent pulmonic valve was successful operated upon without insertion of the pulmonic valve. The narrow pulmonic valve annulus was enlarged with a transannular pericardial patch graft. The postoperative course was uneventful.