Lipoprotein glomerulopathy is a disease characterized by intraglomerular lipoprotein thrombi and abnormal lipid metabolism similar to type III hyperlipoproteinemia. Recently, glomerular lesions compatible with lipoprotein glomerulopathy were found in a 38-year-old man. In this case, plasma apolipoprotein (apo) E level was elevated, and the apo E isoform was heterozygous E2/3, similar to most of the reported cases of lipoprotein glomerulopathy. However, the plasma levels of triglyceride and total cholesterol were within their respective normal ranges. Review in 11 cases revealed that lipoprotein glomerulopathy was not always associated with hyperlipidemia, and that apo E abnormality was always recognized. These findings suggest that lipoprotein thrombi associated with lipoprotein glomerulopathy are not induced via systemic hyperlipidemia but may be formed by intraglomerular accumulation of lipoprotein composed of abnormal apo E.