A very rare combination including tetralogy of Fallot (TOF), absent pulmonary valve, and isolated left pulmonary artery is described. A 4-month-old girl presented with mild cyanosis and severe dyspnea. Echocardiography established the diagnosis of TOF and absent pulmonary valve with severe pulmonary regurgitation and moderate stenosis at the pulmonary valve site. A right ventricle angiogram showed the severe aneurismal dilation of the proximal right pulmonary artery and the remarkable pulmonary annular stenosis but failed to show the left pulmonary artery (LPA). An angiography after pulmonary vein wedge injection demonstrated the isolated and hypoplastic LPA.