One case of anomalous origin of the right pulmonary artery from ascending aorta is reported. The anomalies in the origin of the main pulmonary branches are classified in Type A: Anomalous origin of a pulmonary artery from ascending aorta; Type B: Anomalous origin of a pulmonary artery from the aortic arch through a ductus arteriosus, and Type C: Absence of a pulmonary artery with pulmonary vasculariation through systemic arteries arising from descending aorta. The normal embryology of the 6th. aortic arch and the pulmonary branches and the differents theories postulated about the embryopathogenesis of these malformations are reviewed. The outstanding clinical, hemodynamic, angiocardiographic and surgical features are also discussed.