Coronary artery aneurysms rarely develop in patients with systemic lupus erythematosus. We herein describe a case of a right coronary artery aneurysm associated with systemic lupus erythematosus. A 49-year-old woman with known systemic lupus erythematosus presented with an acute myocardial infarction. A coronary artery aneurysm and thrombo-occlusion in the right coronary artery necessitated percutaneous coronary intervention. She experienced three myocardial infarctions during the following two months. A coronary artery dissection occurred during the percutaneous coronary intervention at the time of the last myocardial infarction, and emergency coronary artery bypass grafting was successfully performed. Difficulties in treatment with percutaneous coronary intervention and the fact that arteritis is a possible cause of a coronary artery aneurysm may indicate that surgical therapy, including coronary artery bypass grafting with or without the obliteration of an aneurysm, is the treatment of choice for a coronary artery aneurysm with systemic lupus erythematosus.