Lipoprotein glomerulopathy (LPG) is a newly recognized renal disease of lipid deposition in the glomerular and abnormal lipoprotein profiles similar to those in type III hyperlipoproteinema, and eventually most of the patients would progressive to end-stage renal disease( ESRD). LPG is characterized by a peculiar histology, in that it exhibits thrombus-like lipoproteins in the glomerular capillaries, and which the diagnosis  must depend on renal biopsy, so it has lower diagnostic and to date all the cases have been reported less than 100 in the global. Now the mechanism of LPG has not been excluded clearly, increasing evidences have been shown to be associated with some apolipoprotein E (ApoE) mutations, and some other pathogenic factors including dyslipidemia, oxidative stress, mechanism of renal situ. This article  review the epidemiology , clinical manifestation , histopathology , laboratory  examination, importantly to discuss the pathogenesis of LPG .