Abstract: Interrupted aortic arc is defined as a loss of luminal continuity between the ascending and descending aorta. It is a rare congenital malformation and it is classified on the basis of the branches that originate from the proximal aorta. An interrupted aortic arc is usually associated with another congenital intracardiac malformation and patients with isolated interrupted aortic may survive to advanced age. Here we report a very rare case of an isolated interrupted aortic arc in a 75-year-old woman who presented with acute myocardial infarction.