An exceptional case is reported of hyper IgE syndrome and membranoproliferative glomerulonephritis with onset in 1964. Renal disease, symptoms of hyper IgE syndrome and level of serum IgE were improved by a long-term cyclosporine treatment (3/5 mg/kg/day) with a follow-up of 4 years. The characteristics of the hyper IgE syndrome, described by Buckley in 1972 are given and the respective roles of IgE-BF, IL4 and interferon in the mechanism of increase in serum IgE is discussed.