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Acute necrotizing encephalopathy of childhood (ANEC) is a disease characterized by respiratory or gastrointestinal infection and high fever accompanying with rapid alteration of consciousness and seizures. Acute encephalopathy following viral infection with seizure and altered of consciousness, and absence of CSF pleocytosios with occasional increased level of protein are clinical characteristics of ANEC (1). This disease is nearly exclusively in East Asian infants and children who had previously been completely healthy (2). Serial magnetic resonance imaging examinations have demonstrated symmetric lesion involving the thalami, brainstem, cerebellum, and white matter in this disease (8). The condition accompanies a Poor Prognosis With high morbidity and mortality rates. Our cases were A 5 years old boy, A 4years old girl and A 3 years old girl With ANEC respect to Bu Ali Hospital in Ardabil.