Abstract: The concentration of nigh-density lipoprotein subfractions and the level of lipid peroxides were determined in four cases of Fabry's disease in two families.The results showed that the concentration of H.DL2-C decreased,the HDL3-G/HDL2-C ratio and the content of lipid peroxides in the sera were significantly-elevated in lour patients.Electron xnicrascopy showed that there were different kinds of electron dense inclusion in endothelial cells,pericytes,smooth muscle cells and febroblast.In the cytoplasra of these cells,there were round lipid granules in different sizes.This study suggests that the pathogenesis of Fabry's.disease may be caused by the accumulation of sphingolipids and metabolic disorder of HDL-C subfractions (HDL3-C and HDL2-C) and lipid peroxides.