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Kawasaki disease is a systemic vasculitis of unknown etiology. The most significant sequela of acute Kawasaki disease are related to information of small to medium sized arteries and, in particular, the development of coronary artery dilation and aneurysms (CAA). The aim of this study was to assess the cardiovascular complications of Kawasaki disease in Iran. The studied population consisted of 54 patients who were diagnosed to have Kawasaki disease and referred for cardiac evaluation. There were 30 boys and 24 girls, aged between 5 months and 13 years (Mean 3.9 years). All patients except 3 had received standard treatment with high-dose Aspirin and intravenous ? Globulin (IVGG). All patients were evaluated by physical exam, chest X-ray, ECG and echocardiography (ECHO). Abnormal cardiac findings included: Cardiomegaly in 4 (7.6%), ECG changes in 4 (7.6%), Mitral regurgitation in 3 (5.5%) LV dysfunction in 2 (3.7%), pericardial effusion in 4 (7.6%) and coronary artery dilatation in 5 (9.2%). Conclusion: 1) Kawasaki disease and its cardiovascular complications are not uncommon in Iran. 2) Coronary artery involvement is a major problem. 3) IVGG therapy reduces the risk of cardiac complications. 4) ECHO is a valuable tool in acute phase and follow-up period.