Objective To investigate the important clinical features and prognosis of febrile infection-related epilepsy syndrome （FIRES）. Methods A retrospective analysis was performed for the data of 15 children with FIRES who were hospitalized and treated in Peking University First Hospital from March 2022 to June 2024，including clinical features，treatment regimens，and prognosis，and follow-up was performed by telephone. Results The median duration of status epilepticus was 15 days for all children. Of all 15 children，14（93.3%） were comorbid with disturbance of consciousness，8（53.3%） were comorbid with respiratory failure and underwent endotracheal incubation，and 13（86.7%） had been admitted to the intensive care unit. In the acute stage，7 children underwent the examination of various inflammatory factors in blood and cerebrospinal fluid，including interleukin（IL）-1β，IL-2，IL-4，IL-5，IL-6，IL-8，IL-10，and tumor necrosis factor-α，and all 7 children had significant increases in the levels of inflammatory factors in cerebrospinal fluid，which were significantly higher than the levels of inflammatory factors in serum. Of all 15 children，12（80%） had diffuse slow wave changes on electroencephalography，and migrating focal seizures were detected in 7 children（46.7%）. Cranial magnetic resonance imaging（MRI） manifestations in the acute stage included temporal and insular cortical edema（60%），abnormal white matter signal（33.3%），and claustrum sign（13.3%），and MRI features in the chronic stage included the deepening of cerebral sulci（75%） and ventricular dilatation（33.3%）. The treatment in the acute stage included intravenous drip of gamma-globulin and high-dose methylprednisolone in 15 children（effective in 2 children），ketogenic diet in 4 children （effective in 1 child），tocilizumab in 5 children（effective in 3 children），and anakinra in 2 children（effective in 1 child）. As of the last follow-up，the median duration of disease was 14.0 months（4-65 months） for all patients，and only 2 children achieved complete seizure control，while the remaining 13 children had refractory epilepsy. Cognitive impairment was observed in 93.3% of the children. Conclusion FIRES often has acute and severe conditions，and first-line immunotherapies often have a poor therapeutic effect. Tocilizumab and anakinra may be effective in some patients with seizures in the acute stage.