Tetralogy of Fallot with absent pulmonary valve is a rare congenital heart defect which includes all the findings of Tetralogy of Fallot with an underdeveloped or total absence of the pulmonary valve tissue. It is reported in about 3% of all Tetralogy of Fallot cases. The pulmonary valve annulus is usually narrowed and the lack of pulmonary valve tissue generates severe pulmonary regurgitation. The to-and-fro blood flow across the right ventricular outflow tract produces significant dilatation of the right ventricle and the main and branch pulmonary arteries. Patients will frequently present postnatally with respiratory distress secondary to severely dilated branch pulmonary arteries which place extrinsic compression on the trachea. Tetralogy of Fallot with absent pulmonary valve is rarely associated with a patent ductus arteriosus and often occurs with chromosomal abnormalities.