Objective To explore the clinical characteristics of children with enterovirus associated encephalitis, in order to provide some help for clinicians to understand the disease. Methods The clinical data of 37 children with enterovirus related encephalitis treated in the infection department of Guangzhou Women and Children's Medical Center from January 2018 to December 2019 were analyzed retrospectively. Results There were 28 males and 9 females, with a male to female ratio of 3.11:1. The main clinical symptoms and signs were fever (97.30%), oral herpes and/or rash (54.05%); the common nervous system symptoms were vomiting (56.76%), headache (56.76%) and convulsion (29.72%). There were significant differences in convulsion, vomiting and headache symptoms between ≤ 1 year old group and other age groups. The leukocytes level in cerebrospinal fluid was elevated. All children recovered and discharged without neurological sequelae. Conclusions Nearly half of children with enterovirus associated encephalitis had no clinical manifestations of pharyngeal herpes or rash. The main manifestations of children under 1 year old were fever and convulsion. The main manifestations of children over 1 year old, especially preschool and school-age patients, were fever and vomiting with headache. Early diagnosis and treatment had good prognosis.