A 55-year-old man developed acute myocardial infarction (AMI) related to a large coronary artery aneurysm and a distal coronary stenotic lesion after steroid therapy for systemic lupus erythematosus (SLE). Only 13 SLE patients with AMI caused by coronary artery aneurysms have been reported, 11 of whom were young or middle-aged women and the 2 remaining were young men. This is the first report of a middle-aged man with multiple coronary lesions.