We reported a case where scintillating scotoma was the first and only symptom of systemic lupus erythematosus. The patient was diagnosed as having classic migraine and was given cafergot and phenytoin with no effect. During this treatment, severe myocardial infarction occurred and SLE was found to be the underlying disease responsible for scintillating scotoma. Scotoma attacks disappeared by steroid treatment but his general condition remained poor.