A previously normotensive 9-year-old boy was admitted to the Pediatric Emergency Department with bilateral periorbital edema and gross hematuria for 24 hours. 20 days before, he had experienced an episode of mild sore throat spontaneously recovered. On admission general conditions, neurological and visual examinations were normal except for periorbital edemas. Axillary temperature was 36.9°C, oxygen saturation 98%, heart rate 88 beats per minute, body weight 35 kg (two more than the previous week), and body height 1.350 m. Blood pressure was increased with systolic values ranging between 125 and 130 mmHg and diastolic values ranging between 80 and 85 mmHg (stage 1 hypertension: 95th percentile to the 99th percentile for gender, age, and height plus 5 mmHg []). Blood and urine investigations were consistent for acute renal insufficiency due to a nephritic-nephrotic syndrome. Since the 2nd day of hospitalization, the patient was treated with one bolus of methylprednisolone (15 mg/Kg) per day associated with strict blood pressure monitoring. On the 4th day of hospitalization, the patient developed severe headache associated with increased blood pressure up to 150/120 mmHg (stage 2 hypertension: > 99th percentile for gender, age and height plus 5 mmHg []). Alterations of conscious level, visual symptoms and vomiting were not present. Furthermore, cognitive dysfunctions, visual field defects, sensory abnormalities and ataxia were absent on the neurological examination.A brain MRI showed in fluid attenuated inversion recovery imaging (T2 coronal) high signal intensities in cerebellar white matter and in parasagittal subcortical parietal regions. Diffusion weighted images did not show any restriction of diffusivity. Methylprednisolone was discontinued. Candesartan was initiated and on 6th day of hospitalization, blood pressure normalized and both headache and periorbital edemas resolved. On 9th day of hospitalization, a control MRI showed complete regression of all the abnormalities. Arterial hypertension, MRI findings and clinical outcome were consistent with the diagnosis of PRES. The child was discharged in good clinical condition without any further treatment. 3 weeks later, clinical examination was unremarkable and blood pressure 105/70 mmHg. Complement C3 and serum creatinine normalized and urinalysis revealed only persistent isolated microscopic hematuria (7 cells per high-power field).