Kawasaki disease (KD) was first described by Dr. Tomisaku Kawasaki in his 1975 study, published in Pediatrics. Its pathogenesis is still not clearly understood. Early diagnosis and treatment are very important to preventing concomitant coronary artery complications. Most KD patients respond well to the standard treatment of aspirin and intravenous immunoglobulin; however, some of them are refractory to the standard treatment, and so adjuvant therapies with corticosteroids and anti-tumor necrosis factor-α (TNF-α) antibody are necessary. In this article, the author reviews and summarizes the most recent literature on the treatment of refractory KD.