Abstract: Interrupted Aortic Arch (IAA) is a rare congenital malformation of the aortic arch that occurs in 3 per million live births. This anomaly is defined as a loss of luminal continuity between the ascending and descending portion of the aorta that entails a very poor prognosis without surgical treatment. In most cases IAA is associated with an intracardiac malformation such as VSD, PDA, bicuspid aortic valve, left ventricular outflow tract obstruction or aorto-pulmonary window We report a 16 years old boy with IAA associated with double inlet left ventricle, aneurysmal dilatation of pulmonary artery, giant PDA and severe pulmonary hypertension. As our knowledge there is no previous report of IAA associated with complex congenital heart disease such as our case.