Introduction: Atypical Kawasaki disease (KD) is a diagnostic dilemma. Acute acalculous cholestasis and liver involvement sometimes indicate atypical presentations of KD. Case Presentation: We report a four-year-old boy with fever, abdominal pain, vomiting, jaundice and mild liver enlargement who gradually developed other signs of KD. He was treated by aspirin, intravenous immunoglobulin and finally methylprednisolone pulse therapy with no sequela. Conclusions: KD should be kept in mind as one of the causes of childhood acalculous cholestasis, abdominal discomfort and prolonged fever.