The syndrome of tetralogy of Fallot with absent pulmonary valve is characterized by aneurysmal dilatation of the pulmonary arteries causing tracheobronchial obstruction of varying degree. Relief of this obstruction is the main goal of the surgical repair and can best be achieved by appropriate pulmonary arterioplasty. We describe our current technique to repair this syndrome in infants and older children including pulmonary arterioplasty, ventricular septal defect closure, and right ventricular outflow tract reconstruction without valve insertion.