Severe posterior reversible encephalopathy syndrome resolved with craniectomy.

Posterior reversible encephalopathy syndrome (PRES) has been thought to be a benign disease, but recently severe cases have been reported with increasing recognition. A 3-year-old girl with congenital nephrotic syndrome had rapidly progressed to coma. Computed tomography (CT) of the head showed striking swelling of the brainstem and transtentorial herniation. Emergency decompressive craniectomy was performed. Consecutively, blood pressure was optimally controlled. The patient gradually recovered to the previous state before onset of PRES. Rapid improvement of clinical symptoms and rapid resolution of abnormal findings on serial CT led to diagnosis of PRES. In severe PRES with unstable vital signs, surgical intervention should be considered as well as appropriate blood pressure management.