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Posterior reversible encephalopathy syndrome (PRES) is a condition characterized by headache, altered mental status, seizure and visual dysfunction, and transient motor deficit. The incidence of PRES in systemic lupus erythematosus (SLE) has not been clearly known. Risk factors for PRES in SLE include hypertension, renal failure, cytotoxic drugs, and active lupus. We describe a patient of SLE with nephritis on cyclophosphamide monthly pulse that developed PRES and, unfortunately, succumbed to her illness.