The absent pulmonary valve syndrome consists of a severely hypoplastic pulmonary valve with anular stenosis, aneurysmal dilatation of the main pulmonary artery with one or both pulmonary branches also dilated, and a ventricular septal defect. From 1955 to 1975, 15 patients were seen at Texas Children's Hospital with this syndrome. Although anatomically similar to tetralogy of Fallot, the most significant symptoms during early infancy were secondary to bronchial compression resulting from the dilated pulmonary arteries and enlarged left atrium. Intracardiac repair performed on two infants at four months of age failed to ameliorate the respiratory symptoms and both died. Seven patients from 2.3 to 16 years of age were operated upon, with one late postoperative death. Five other patients have not yet received surgical intervention. We recommend vigorous continual respiratory therapy for for infants and small children and intracardiac repair in older children since repair in infancy does not relieve respiratory symptoms. We do not recommend a prosthetic pulmonary valve unless there is persistently elevated main pulmonary artery pressure.