Abstract: Febrile infection-related epilepsy syndrome(FIRES) is a rare and catastrophic epileptic encephalopathy that mostly occurs in healthy school-age children and progresses rapidly and it causes severe neurological dysfunction, chronic epilepsy and has a high mortality rate. FIRES was difficult to treat and patients had a poor prognosis. The exact pathogenesis of FIRES is unclear and may be related to fulminant neurogenic inflammation in the brain. Recently, more and more studies have shown that IL-1β/IL-1R1 axis signaling pathway may play an important role in FIRES. The mutation of interleukin-1 receptor antagonist gene(IL1RN) may have regulation effect on the occurrence of FIRES to some extent. Clinically, anakinra, an interleukin-1 receptor 1(IL-1R1) antagonist, may help control seizures in FIRES patients. The purpose of this review was to explore the pathogenesis of FIRES based on the IL-1β/IL-1R1 axis signaling pathway and to provide theoretical support for clinical diagnosis and drug development based on this pathway.