Abstract: Collagenofibrotic glomerulopathy （CG）, also called Collagen Type Ⅲ glomerulopathy, which is a rare non-immune-mediated primary glomerular disease, characterized by abnormal accumulation of type Ⅲ collagen fibrils within the mesangial matrix and subendothelial space in the glomeruli. The major clinical manifestations are proteinuria, peripheral edema, hypertension and occasional progression to end-stage renal disease. The diagnosis is mainly based on electron microscopy. There is no effective treatment, and the overall prognosis is poor. For patients with end-stage renal disease, kidney transplantation may have a better prognosis.