The association between persistent truncus arteriosus and interrupted aortic arch is a rare but important condition since it has special clinical and therapeutic implications. In this article we present three cases with this malformation. The clinical characteristics are similar to patients with isolated truncus such as heart failure and cyanosis since birth, a protosystolic click, an ejection murmur over the left parasternal line, heart enlargement, increased pulmonary vascular markings and biventricular hypertrophy. The clinical suspicion of interrupted aortic arch is bases on the presence of asymmetric pulses in the upper and lower limbs, but this difference is present only when the arterial duct is restrictive. Because of that, the echocardiographic analysis, mainly, and the angiocardiogram, secondarily, are very important diagnostic tools. We analyze the echocardiographic diagnostic findings, as well as the angiocardiographic anatomy. Also, some important pathophysiological aspects are discussed.