Kawasaki disease (KD) is one of the most common childhood vasculitides and may lead to coronary arterial complications. KD has been reported in more than 60 countries over five continents. Previous publications have provided a comprehensive description of the epidemiologic features of KD including incidence, age of onset, seasonal trends, and rates of cardiac lesions. However, the interactions among the KD patients, time (seasons) and place have been less well studied. We review the current global epidemiology of KD and focus on the longitudinal changes in incidence, seasonality and response to intravenous immunoglobulin (IVIG) therapy.