SummaryCoronary artery ectasia (CAE), a discrete or fusiform arterial dilatation, is an uncommon angiographic finding. We report the case of a patient presenting with an acute coronary syndrome in whom further evaluation revealed coronary artery disease with severe CAE in the presence of an abdominal aortic aneurysm (AAA). Since both entities are strongly associated with local and systemic atherosclerosis, they have traditionally been viewed as a variant of atherosclerosis. The combined occurrence of CAE and AAA, as in the present case, raises questions about common pathogenetic mechanisms apart from atherosclerosis. The respective evidence will be reviewed.