Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in infants and young children. Initially described in 1967 by Dr. Tomisaku Kawasaki, it is now the most common cause of acquired heart disease among children in developed nations. Although KD has been reported across all racial and ethnic groups, the incidence of KD is more common among Asians, which suggests differences of race-specific susceptibility. The prevalence of the disease varies considerably among different Asian countries, and there is a higher rate of KD reported in Asian countries such as Japan and Korea than in other countries. In Korea, a nationwide epidemiological study has been conducted every three years since the 1990s by the Korean Pediatric Heart Association to determine the epidemiologic patterns and incidence rate of KD in Korea. It was thus found in a recent survey (2003-2005) that the average annual incidence of 105.0/100,000 Korean children under the age of five years was the second-highest reported rate in the world, after Japan.