C3-glomerulopathy includes several nephropathy charac-terised by almost only C3 deposits without immunoglobu-lins with a mesangial proliferative or membrano-proliferative pattern on light microscopy. C3-glomerulopathy is determined by a dysregulation of the al-ternative pattern of complement due to autoantibodies such as C3 nephritic factor or mutations of complement pro-teins. Corticosteroids combined with mofetil micofenolate could be effective in inducing a partial or complete remis-sion. Eculizumab, the humanised monoclonal antibody di-rected against C5, has been shown to have a favourable effect in several case series of patients.