Intravenous immunoglobulin (IVIG) have been widely used in clinical practice for more than 35 years. Their specificity and diversity and their relative safety make them potent therapy in antibody deficiencies, certain infections and several autoimmune and inflammatory disorders. IVIG is a biological drug that is used routinely for idiopathic thrombocytopenic purpura, Kawasaki disease, and Guillain-Barré syndrome. Therapeutic approaches for autoimmune diseases are primarily based on suppressive measures that down regulate an over productive immune system. IVIG efficacy has been established in many clinical trials for various autoimmune diseases, vasculitis and neuroimmunological, dermatological and hematological disorders. Despite the evidence of efficacy, there are no generally accepted therapeutic guidelines, the dosage and timing of IVIG therapy, and questions of costs/benefits ratio still remain insufficiently documented and multicentric controlled clinical trials are necessary. Most available evidence for a benefit for IVIG in children comes from uncontrolled open series or case reports.