Kawasaki disease is a systemic vasculitis of unknown aetiology that has been reported worldwide since its initial description in Japanese children. The most significant sequelae of acute Kawasaki disease are related to the inflammation of small to medium sized arteries and, in particular, the development of coronary artery aneurysms. Because the aetiology is unknown, pharmacological therapy is nonspecific and directed towards modulation of the inflammatory response and inhibition of platelet activation with the aim of preventing coronary artery aneurysms. In the US, the recommended treatment for Kawasaki disease in the acute phase is a single, high dose of intravenous gammaglobulin (2 g/kg) and high dose aspirin (80 to 100 mg/kg/day). Use of this regimen has resulted in a significant decrease in the incidence of coronary artery abnormalities. Although the American Heart Association currently recommends high dose aspirin, moderate doses are used in Japan and the optimal dose of aspirin is not known. There has been renewed interest in the use of corticosteroids in the treatment of acute Kawasaki disease: however, their precise role remains unclear. Newer antiplatelet agents have also shown some promise in the treatment of patients with coronary artery aneurysms. Long term pharmacological therapy consists primarily of anticoagulation in patients with persistent coronary artery abnormalities. In this review, current recommendations for pharmacological therapy in Kawasaki disease are reviewed and some of the controversies in management of this disease, including management of patients who do not respond to initial therapy and the role of corticosteroids in the acute setting, are outlined.