Kawasaki disease is an acute systemic vasculitis predominantly affecting young children. It is due to an abnormal host response to as yet unidentified infectious trigger(s). Kawasaki disease may cause coronary artery damage, long-term cardiovascular morbidity and occasionally mortality, especially if the diagnosis is missed or timely treatment is not given. This is the first of two updates on Kawasaki disease. Here we review recent advances in epidemiology, possible aetiologies, host susceptibility and pathogenesis of this fascinating condition.