Kawasaki disease, predominantly affecting infants and young children, is a systemic vasculitis with an unknown origin. The diagnosis of the disease is based on clinical criteria. Neuroblastoma is the most common extracranial solid tumor seen children under five years of age as Kawasaki disease. The two conditions are rarely reported together. We describe an infant who was initially diagnosed as Kawasaki disease, and thereafter neuroblastoma. It is discussed whether neuroblastoma plays any role in the pathogenesis of Kawasaki disease in this report.