Dravet syndrome (DS), a severe developmental and epileptic encephalopathy often linked to SCN1A mutations, is defined by a profound thermosensitivity, making fever and hyperthermia potent seizure triggers. This review synthesizes evidence-based strategies and expert consensus for the management of fever and vaccination in children with DS. Management diverges from standard pediatrics, prioritizing aggressive pyrexia control through early antipyretics, physical cooling, and prophylactic benzodiazepines. Proactive strategies are also crucial for non-febrile hyperthermia from triggers like hot baths and overexertion. Although vaccinations can precipitate an initial seizure, they neither cause DS nor worsen its prognosis. Immunization remains strongly recommended, with prophylactic antipyretics advised as a key risk-mitigation measure. Importantly, current management strategies are based primarily on expert consensus rather than controlled clinical trials. Bridging expert consensus with clinical evidence is essential to reduce morbidity and improve long-term quality of life in DS.
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