Intravenous immunoglobulin (IVIG) for the treatment of primary immunodeficiency disorders have been administrated for more than 25 years. However, the recognition of the anti-inflammatory and immune-modulatory actions of IVIG resulted broader applications to autoimmunity and systemic inflammatory conditions. The major focus of this review is the usefulness of IVIG therapy in children kidney disease, particularly in severe, atypical hemolytic-uremic syndrome and thrombotic thrombocytopenic purpura, refractory to standard therapy, including plasmaferesis. The role of intravenous immunoglobulins in the treatment and prevention of infection, the most common complication in nephrotic syndrome is also discussed. Probably, in the future, similarly to adults, IVIG will be used for the therapy of some forms of glomerulopathies.