Kawasaki disease is the leading cause of acquired heart disease in infants and young children. Kawasaki disease that manifests as facial nerve palsy is extremely rare, and the diagnosis is challenging. We report a 4-month-old girl with Kawasaki disease who presented with fever, redness and cracking in the lips and oral cavity, and a right facial nerve palsy. The infant received intravenous immunoglobulin, acetyl salicylic acid, and warfarin. The patient’s fever subsided on the following day, and the right-sided facial nerve palsy was relieved a month later.