Brain meningioma, the most common benign brain tumor, has been reported to account for 13-26% of all intracranial tumors, with a crude incidence rate of 2.3 per 100,000 persons for all types of meningiomas. The prevalence of neuropsychiatric lupus erythematosus is 15-91% and its clinical manifestations are diverse: from mild cognitive dysfunction to serious neurological or psychiatric symptoms. Here, we report the first Korean patient with brain meningioma and systemic lupus erythematosus who had undergone surgical tumor resection.