While there is a generally accepted standard approach to the management of Kawasaki disease (KD) in North America, controversy still exists regarding certain aspects of treatment. Do all patients require treatment with intravenous immunoglobulin (IVIG)? What is the appropriate dose of aspirin (ASA) during the acute phase of the disease? Is there a role for corticosteroids in those who fail IVIG? How should patients with atypical, incomplete or late presentations of KD be managed? What is the appropriate long-term management and follow-up, particularly for those without coronary artery abnormalities (CAA)? Is there a role for surgical intervention, particularly transplantation? These questions, among others, are explored with reference to the pertinent literature.IVIG has been well studied and shown to be efficacious in a number of studies and in two meta-analyses, with clear evidence to support the use of 2g/kg in a single dose. The appropriate dose of ASA during the acute phase is less clear but, increasingly, data suggest that lower doses of ASA are adequate and perhaps more appropriate. Corticosteroids appear to have a role in those who have failed IVIG but this requires further study before being embraced as accepted treatment. The management of less typical presentations of KD remains controversial, with inadequate data to direct us, although there is a general trend towards treating such patients with IVIG. Careful follow-up of all patients is recommended and, while there are guidelines for this, there is no clear consensus on the most appropriate monitoring investigations for those with and without CAA. There is an expanding role for transplantation, with clearly defined indications for this intervention.