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Kawasaki disease is an acute vasculitis that occurs especially in young children. Because there is no specific laboratory test available, diagnosis has to be made on the basis of clinical characteristics: prolonged fever, oropharyngeal changes, conjunctival injection, erythema and edema of hands and feet, rash, and cervical lymphadenopathy. Without treatment there is a 25% chance of cardiac complications, especially aneurysms of the coronary arteries. Early treatment with intravenous immunoglobulin reduces this risk to 5%. Accurate diagnosis and therapy is crucial. Kawasaki syndrome has been reported in all racial groups with the highest incidence in Japanese children. Together with the fact that the disease is more common in boys this indicates that genetic factors play an important role in determining susceptibility to a (probably infectious) trigger. In spite of 40 years of intensive scientific research, the cause of Kawasaki disease still remains unknown.