Posterior reversible encephalopathy syndrome (PRES) was described in adults more than children. It was reported in cases of nephrotic syndrome which were mainly on immunosuppressant medications or had severe hypertension. We report the case of a 9-years old girl who presented with nephrotic syndrome and moderate hypertension. Nine days after the initiation of steroid therapy she developed disturbed level of consciousness and generalized tonic clonic seizures. Her blood pressure was 145/90 mm Hg

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... and she had normal serum creatinine and electrolytes levels. She was treated with furosemide and convulsions were controlled. After regaining consciousness, the patient complained of loss of vision. Fundal examination was normal. Magnetic resonant imaging, axial FLAIR images and diffusion weighted imaging showed hyper-intensity signal in the parietooccipital areas. Magnetic resonant arteriography and spectroscopy excluded ischemic insults and neoplastic process. She regained full consciousness and normal vision and was discharged from the ICU four days later. PRES was diagnosed based on the typical pattern of brain imaging and the reversibility of symptoms. Nephrotic syndrome in children should be considered a risk factor for developing PRES even without the use of immunosuppressant agents or high doses of steroid.