The origin of the right pulmonary artery from the ascending aorta is a rare cogenital anomaly, with very few reports in the literature. We describe two cases of this rare malformation, one of them, associated with interruption of the aortic arch (type B). In both cases, the diagnosis was made by two-dimensional echocardiography, with angiographic confirmation in one of them. The origin of the right pulmonary artery was close to the aortic valve, anomaly pathogenetically distinct from the type that arises close to the innominate artery.