The authors report the case of a 50 year old man with pseudowanthoma elastica with a history of myocardial infarction and severe aortic regurgitation. Angiography showed multiple coronary artery aneurysms and aneurysmal dilatation of the aortic annulus. The outcome after triple coronary bypass surgery with aortic valve replacement in a valved Bentall conduit was favourable. Pseudoxanthoma elastica is a rare condition in which the prognosis depends on the degree of vascular involvement. In this context, coronary artery aneurysms and aneurysmal dilatation of the aorta are rare complications.