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Background: Kawasaki disease is one of the leading causes of acquired heart disease in children. It is an acute self-limited vasculitis that predominantly affects infants and children younger than 5 years of age. These patients present with nonspecific symptoms, such as fever and lymphadenopathy, making the diagnosis challenging. This disease can have serious and potentially fatal outcomes, and prompt recognition of this disease is vital to the patient’s outcome. We present a complete review of the disease, including the epidemiology, pathophysiology, diagnosis and management of acute Kawasaki disease, the natural history of this disease, and follow up of these patients as they transition into the adult cardiology practice. Methods: Our systematic review information were collected from articles retrieved from PubMed library. Keywords that were used included; Kawasaki disease, coronary artery disease, coronary artery aneurysm, pediatric coronary artery disease, epidemiology of Kawasaki disease and treatment of Kawasaki disease. We included only relevant to the topic articles. No exclusion criteria were applied. Conclusions: Kawasaki disease incidence tends to be increasing over the last decade in the united states. Seasonality of the disease has been described in Japan. It is a mysterious disease with unknown etiology, however, multiple hypotheses have been proposed and tested to explain the pathophysiology. As this disease has an associated high morbidity and mortality, prompt recognition and management of this disease is important to the patient’s overall prognosis and survival.