Abstract: Objective To investigate clinical characteristics,treatment and prognosis of acute necrotizing encephalopathy （ANE） in children. Methods Three pediatric cases of ANE were reported. Literature review was conducted from PubMed,Web of Science,CNKI and Wanfang Data in recent 10 years by using the searching word of “acute necrotizing encephalopathy” in both Chinese and English. Clinical data of children with ANE under the age of 14 who had atypical radiographic features were collected and analyzed. Results All three pediatric cases of ANE presented with fever and convulsion as the initial symptoms. CT scan of the head revealed cerebral edema alone. The patients died after rapid progression into multiple organ dysfunction syndrome （MODS）. After literature review,19 pediatric cases of ANE with atypical radiographic features were collected. Clinical efficacy of combined use of high-dose glucocorticoid （hormone） shock,intravenous infusion of human immunoglobulin,plasma exchange,antiviral and targeted therapy remained elusive. A majority of survivors developed sequela. Conclusions ANE is a rare disease with rapid progression,extremely high mortality,poor prognosis and unknown pathogenesis. No effective treatment is available. Previously,diagnostic criteria of ANE are determined based on typical MRI features,which are not in accordance with clinical practice and may delay the diagnosis and treatment. Therefore,for children with acute brain dysfunction in seasons when viral diseases are prevalent,it is necessary to lift restrictions on imaging diagnostic criteria,or develop a new scoring diagnostic criterion combined with clinical practice,which is conducive to prompt clinical diagnosis and active intervention and treatment for ANE. Clinicians should deepen their understanding of ANE and avoid missed diagnosis and misdiagnosis.