Treatment of Kawasaki disease (KD) with intravenous immunoglobulin (IVIG) administered within the initial 10 days of fever onset decreases the risk of coronary artery aneurysms (CAAs) from ∼ 25% to less than 5%. However, patients with IVIG resistance, young infants, men, highly inflamed patients, and/or those with coronary changes at diagnosis remain at high risk for CAA. High-risk patients may benefit from acute, adjunctive antiinflammatory treatment in addition to IVIG. Optimal therapy remains unknown. This article reviews the acute pharmacologic management of patients with KD, focusing on adjunctive primary therapy options and treatment of patients with IVIG resistance.