Acute myocardial infarction is an important and sometimes fatal complication of systemic lupus erythematosus (SLE). We describe a case of acute myocardial infarction in a 23-year-old woman with SLE. Angiography revealed coronary ectasia in the left main and proximal circumflex coronary arteries, as well as a stenotic lesion in the left anterior descending artery. The possible pathophysiology is discussed.