Background: Distal segment atresia (isthmus) is an extremely rare anatomical variant of obstructive aortic arch anomalies.
Case Report: We present the case of a newborn who, at 48 hours of life, presented a clinical picture of heart failure. The initial echocardiogram showed a congenital interrupted aortic arch type A, patent ductus arteriosus, and ventricular septal defect.