Background: Kawasaki disease (KD) is idiopathic autoimmune self-limiting disease. It was discovered by Professor Tomisakan Kawasaki in Japan in 1967. it is the second wide spread vasculitis disease in pediatric. Objectives: The present review is aimed to high light on the symptoms, epidemiology, etiology, pathology, complication, cardiac manifestation, diagnosis, and treatment of Kawasaki disease. It impacts children younger than 5 years old and is common in boys than girls. 85% of children KD younger than 5 years, peak age between 18-24 months. It is considered as common cause of acquired heart disease in children. It is widely spread in developed countries, and it is common in developing countries, such as China, Japan, Korea, and Taiwan. It is remarkable in summer to spring seasons of year particularly in China and Korea, winter and spring seasons in Europe and Australia. It is result from genetic and pathogenic causes by stimulation of immune system extra or over than normal response of body to bacterial or virus antigens. In all KD cases histological appearance like as myocarditis and fibrosis. It is a significant due to cardiac complications such as development of coronary abnormality in 25% of misdiagnosed groups, and 3-5% in resistance groups which treated by Intravenous immunoglobulin (IVIG). Several clinical and laboratory manifestation rise risk of coronary artery abnormality which including fever for long duration, boys, old ages, prolonged increase levels of ESR and CRP, increasing count of white blood cells, normocytic normochromic anemia, hypoalbuminemia, and thrombocytopenia. Cardiac features of Kawasaki disease include heart failure, ECG abrormality, pericarditis, myocarditis, plural effusion, myocardial infarction, valvular incompetence. The purpose of management is decrease inflammation of cardiac cells and cells of coronary artery lining during acute period of illness, and prevent thromybosis formation in coronary artery by intravenous immunoglob