Children frequently have neurological issues such febrile encephalopathy and fever-related seizures. The majority of cases are caused by infections of the neurological system. The trifecta of encephalopathy, fever, and seizures is also associated with a range of inflammatory and infectious diseases. In addition to complex febrile seizures and febrile status epilepticus, age-related clinical phenotypes of fever-related epilepsy and encephalopathy include reversible splenial lesion syndrome (RESLES), infantile hemi convulsion hemiplegia epilepsy syndrome (IHHE), fever infection related epilepsy syndrome of childhood (FIRES), acute necrotizing encephalopathy of childhood (ANE), acute encephalopathy with delayed diffusion restriction (AESD). For an accurate diagnosis and the quick application of immunomodulatory/ immunosuppressive therapy, awareness of these entities is crucial. We examine the pathogenesis, clinical manifestations, and therapeutic strategies of these feverrelated encephalopathy and seizure disorders in this review.