Acute myocardial infarction (AMI) is a major cause of death in a patient with systemic lupus erythematosus (SLE). Due to their chronic inflammatory state, patient with SLE has an increased risk of developing coronary artery disease. We report a case of a middle-aged woman with an acute myocardial infarction (AMI) caused by a right coronary artery (RCA) stenosis complicated with severe coronary artery spasm. Our patient has a history of long-standing SLE. Clinical expression of coronary artery disease (CAD) in SLE is the result of different pathophysiologic mechanism. From this case, we raise the importance of the clinician to be aware of the diverse pathophysiologic pathways involving a coronary artery in a patient with SLE.