: Kawasaki disease (KD), a systemic inflammatory disorder with medium-sized vasculitis, mostly occurs among children < five years of age. The highest incidence of KD is among the Asian population. The primary treatment of KD is intravenous immunoglobulin (IVIG) administration. Extreme irritability is a common neurologic manifestation among infants, and central nervous system involvement is uncommon and occurs as a result of vasculitis or IVIG administration late in the treatment course. Here, we discussed an eight-year-old girl with a final diagnosis of atypical KD, who was primarily diagnosed as aseptic meningitis. The patient was admitted with a history of fever, headache, and vomiting and later developed strawberry tongue, coronary involvement, and fingertip desquamation during the follow-up period. Aseptic meningitis, as the first clinical manifestation, made the definitive diagnosis of the KD challenging.