Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinico-radiologic syndrome, first described by Hinchey, et al in 1996, which was reported to be associated with several medical conditions, including hypertensive encephalopathy, chronic renal insufficiency, blood transfusion and eclampsia. RPLS is also reported to be developed during treatment with immuno-suppressive drugs such as cisplatin, cyclosporin A, tacrolimus, and interferon-α, the acute phase of autoimmune disease, and post-transplantation state. Clinical manifestations of RPLS are headache, nausea, vomiting, altered mental status, seizures, cortical blindness, other visual disturbances, and motor deficits. Neuroimaging shows bilateral subcortical and cortical edema with a predominant posterior distribution. The association of RPLS and systemic lupus erythematosus has been rarely reported in the literature, but not yet in Korea. Recently we experienced a patient with SLE, who had RPLS during her disease flare.