Kawasaki disease (KD) is a systemic vasculitis of childhood with a predilection for the coronary arteries. It is the predominant cause of paediatric acquired heart disease in developed countries. The aetiology of KD remains unknown and consequently there is no diagnostic test. The diagnosis is made using a constellation of clinical criteria that in isolation have poor sensitivity and specificity. Early treatment prevents overt coronary artery damage in the majority of children. The long-term effects of childhood KD on later cardiovascular health remain unknown. A recent study showed that treatment of KD in Australia is suboptimal, with late diagnosis occurring in approximately half of the cases and an unacceptably high incidence of acute cardiac involvement. These guidelines highlight the difficulties in the diagnosis of KD and offer some clues that may assist early recognition of this important paediatric disease. They also detail current treatment recommendations and the evidence on which they are based. Increased awareness of the epidemiology and spectrum of the clinical presentation of KD is essential for early recognition and optimal management.