Background: Acute necrotizing encephalopathy (ANE) is a rare acute encephalopathy secondary to infection, which is characterized by convulsion and disturbance of consciousness. Besides, recurrent cases with family history are rarer. To the best of our knowledge, only 12 cases reported were recurrent with family history of encephalopathy in the world. The aim of this study is to report a rare case of recurrent ANE with family history of encephalopathy to provide clinical data for future research of ANE.Case report: A boy had a history of febrile convulsions when he was 1 year old. A few years later, he was hospitalized twice because of convulsions when he was 4 years old and 7 years old. Computed tomography (CT) and magnetic resonance imaging (MRI) were performed on two hospitalizations. Brain CT in the first admission showed low density lesions in bilateral temporal lobes hippocampus and further MRI after 3 days found lesions in bilateral temporal lobe hippocampus, insular lobes, ventrolateral thalamus and pons. Due to the bilateral and symmetrical lesions, metabolic diseases were suspected. When the boy was 7 years old, brain CT was normal while MRI showed multiple lesions in bilateral thalamus, left hippocampus and brainstem. According to the boy's clinical data and MRI findings, pediatrician diagnosed the child as ANE. After 7 days of treatment, he was discharged with slight lameness in the right lower limb.Conclusion: It reports a Chinese child of recurrent acute necrotizing encephalopathy with family history of encephalopathy and reviewed cases similar to him. Diagnosis should be combined with its clinical symptoms, laboratory and imaging results. Clinicians need to improve their understanding in order to achieve early diagnosis and treatment so as to improve prognosis and reduce the occurrence of sequelae.