ObjectiveTo describe the characteristics, differential diagnoses, management and outcomes of severe encephalitis in children.DesignA 10-year retrospective cohort study in children admitted to a tertiary paediatric intensive care unit (PICU) with suspected encephalitis. One to 6 months’ follow-up data were compared between different categories.ParticipantsPatients from 0 to 17 years of age with acute encephalopathy and one or more of fever, seizure, focal neurological findings, cerebrospinal fluid abnormalities, EEG/neuroimaging consistent with encephalitis.Main outcome measuresEpidemiology, clinical features, outcomes and risk factor analysis.Results175 children with encephalitis required intensive care unit (ICU) admission over 10 years. The median age was 4.5 months (IQR 1.6–54.8). The leading cause was enterovirus (n=49, 28%), followed by parechovirus, influenza, herpes simplex virus (HSV), human herpesvirus-6 (HHV-6),Streptococcus pneumoniae, acute-disseminated encephalomyelitis and anti-N-methyl-D-aspartate-receptor-associated encephalitis. Immune-mediated encephalitis had higher prevalence in females, older age and longer duration of encephalopathy. Mechanical ventilation was required by 74 children (42%); haemodynamic support by 28 children (16%), 3 received extracorporeal membrane oxygenation (ECMO) support. Eleven patients died (case fatality rate 6.3%): five with HHV-6, two enterovirus, two influenza, one HSV, one human-metapneumovirus. At follow-up, 34 children had mild or moderate disability, and six severe disability. In a multivariable logistic regression model, three factors were associated with severe disability or death: age <2 years old (OR 8.2, CI 1.0 to 67.2), Herpesviridae aetiology (OR 14.5, CI 1.2 to 177.3) and length of intubation (OR 1.005, CI 1.00 to 1.01).ConclusionsEncephalitis has a varied aetiology and causes death or severe disability in 1 in every 10 children requiring intensive care.