This paper studied the diagnosis and embryology of 20 cases lacking one pulmonary artery out of 2040 patients operated for tetralogy of Fallot. History of severe cyanosis syncope and hemoptysis was usually present in the majority of these patients. The important diagnosis characteristic was asymmetry of plumonary vascularity on X-ray film and the decrease of pulmonary cascular markings on the side without plumonary artery. A right ventricular cardiogram can confirm the absence of one pulmonary artery or the blind-end like change.