Myocardial infarction in patients with systemic lupus erythematosus (SLE) is most commonly a consequence of atherosclerosis. Coronary vasculitis with aneurysms is a rare cause of myocardial ischemia in SLE. We present a case of a 22-year-old woman with a 4-year history of SLE who was admitted with acute onset of chest pain. Although initially treated for lupus pericarditis, she was subsequently found to have an acute myocardial infarction. Cardiac catheterization revealed multiple areas of aneurysmal coronary dilatation and only moderate stenoses of the secondary branches.In view of the angiographic findings, coronary revascularization was not indicated. Anticoagulant therapy was initiated as a result of the presence of large aneurysmal coronary dilatations, which are predisposed to in situ thrombosis and distal embolization. The coronary vasculitis was treated with immunosuppressive therapy. Measures aimed at secondary prevention of coronary artery disease, including optimization of lipid profile, blood pressure control, and prevention of left ventricular postinfarct remodeling, were initiated and continued indefinitely.