Febrile infection-related epilepsy syndrome (FIRES) is a rare neurological condition that typically presents in previously healthy children with sudden-onset frequent seizures following a febrile illness, which often does not have a clear infectious cause. We report a 13-old-year male, a known case of acute lymphoblastic leukemia in remission, presented with fever followed by generalized seizures and encephalopathy. Magnetic resonance imaging brain and cerebrospinal fluid (CSF) examination (including malignant cytology) were normal; electroencephalograph revealed focal convulsive status epilepticus; and CSF interleukin-6 levels were elevated. The diagnosis of FIRES was considered and treatment included mechanical ventilation, multiple antiseizure medications (including infusion of midazolam, thiopentone, and ketamine), methylprednisolone, tocilizumab, and ketogenic diet. He was discharged after 6 weeks with Pediatric Cerebral Performance Category (PCPC) scale of 4 (severe disability) with improvement at 4-month follow-up (PCPC 2, mild disability).