Lipoprotein glomerulopathy (LPG), a rare renal disease, is mainly reported in Japan and China. Chinese cases of LPG showed similar clinical and pathological features as reports from other countries. Three types of APOE mutation have been detected in those patients: APOE Maebashi (142Arg-144Leu-0) and APOE Kyoto (Arg25-Cys) were initially reported, and APOE Guangzhou (Arg150-Pro) is a novel mutation in Chinese patients with LPG. Asymptomatic carriers of all three mutations exist in families, but serum lipid and apolipoprotein E (apoE) levels are markedly elevated. In most of Chinese patients with LPG, long-term treatment with statins or bezafibrates appears to decrease proteinuria. LPG provides a disease model by which to explore pathogenic roles of apoE in common diseases.