Posterior reversible encephalopathy syndrome (PRES) can develop in association with a vast array of conditions including hypertensive encephalopathy and exposure to toxic agents. Clinical manifestations include seizures, headache, focal neurological deficits, visual disturbances and altered mental status and reversible radiological findings such as grey and white matter abnormalities on magnetic resonance imaging. Recurrent PRES in conjunction with periodic lateralized epileptiform discharges on electroencephalogram has been associated with new-onset epilepsy. Here, we report a pediatric case with systemic juvenile idiopathic arthritis with recurrent episodes of PRES and focal occipital epilepsy. The reason for presenting this case is to show an atypical evolution of this disease, rarely described in children.