Since the first report of severe fever with thrombocytopenia syndrome (SFTS) in China, diagnostic methods have evolved. Despite definitive diagnoses, SFTS management has focused on prevention and supportive care. Although conservative treatment is effective for mild cases, there is an urgent need for an effective therapeutic modality to treat severe cases because of the high mortality associated with these. In this study, we report two cases of SFTS with neurological manifestations that fully recovered after a combination treatment consisting of intravenous immunoglobulin and corticosteroid.