We present 5 cases with angiographically absent pulmonary artery: 4 with absence of right pulmonary artery and 1 with absence of the left. We excluded cases of atresia of pulmonary artery, ventricular septal defect and major aorto-pulmonary collaterals. We excluded also cases of anomalous origin of a pulmonary artery from the aorta. Surgical inspection in 3 patients demonstrated total absence of pulmonary main branch (1 case), with intraoperative death, and presence of the hilar portion of the pulmonary artery (2 cases), with successful palliative procedure. It is supportive of efforts to identify as early in life as is feasible the absent vessel, for surgical purposes. In fact the early surgical procedure will ensure development of the vessel and normal lung growth, and will decrease the risk of the following intracardiac repair, when needful.