The posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological entity characterized by the association of clinical neurological signs (headache, confusion, vision changes, vomiting and seizures) and the typical changes in magnetic resonance imaging of the brain.Its pathogenesis is still poorly defined but seems to imply a vascular and endothelial dysfunction. It occurs more frequently in patients with hypertensive encephalopathy, eclampsia, renal failure and has also been associated with the use of immunosuppressive drugs.The authors present a case of PRES in a young woman with systemic lupus erythematosus with active and severe manifestations of the disease.