Kawasaki Disease (KD) is an acute vasculitis of childhood that leads to coronary artery aneurysms in 25% of untreated cases. It is the leading cause of heart disease in children in developed countries. While epidemiologic risk factors have been identified, its etiology remains unknown. Early diagnosis with appropriate intervention has substantially reduced the incidence of cardiac complications, but KD continues to be a leading cause of cardiac morbidity in children. Clinicians need to suspect and recognize the classic and atypical presentations of the disease promptly since early treatment improves the prognosis.