Kawasaki syndrome is a potentially life-threatening disease of early childhood that untreated holds a risk of severe coronary involvement. Its diagnosis is made via a list of clinical signs because etiology and pathophysiology are still unknown and no specific laboratory tool is available. Appropriate therapy with intravenous immunoglobulins and aspirin reduces the incidence of coronary abnormalities to less than 5%. Immunoglobulins have been shown to be highly effective in reducing disease symptoms or their severity and chiefly in reducing the rate of coronary artery aneurysm development. Aspirin is firstly used in high dose for its anti-inflammatory properties and then in low dose for its anti-thrombotic effects. Timely diagnosis and precociously administered treatment are two crucial points in the definition of prognosis for Kawasaki syndrome. In this review heart complications are discussed and therapeutic options stratified according to both severity of coronary involvement and grading of cardiovascular risk.