Kawasaki Disease (KD) is a self-limiting systemic vasculitis common among children under five years of age. Diagnosis of the disease is made primarily from the clinical features presented during the illness. Coronary artery aneurysm (CAA) is the main complication of this disease which can be prevented largely by the early diagnosis and administration of IVIG (Intravenous Immunoglobulin). Even with first dose of IVIG up to 10% -20% of patients develop refractory Kawasaki Disease, i.e., remain unresponsive to initial IVIG treatment. Second dose of IVIG is recommended treatment for refractory Kawasaki Disease but other alternative treatments are also being considered to lower the risk of complications. Different drugs are still in trial phase and some others have limited studies on larger population. Most of the study reports that involved newer drugs have limited patients or are from single centers which are very hard to apply to larger population. We will review general treatment approach in refractory Kawasaki disease.