A 41-year-old Japanese woman with lipoprotein glomerulopathy is reported. The patient presented with nephrotic syndrome, hyperlipoproteinemia and an increased level of plasma Apo E. The damaged glomeruli showed various degrees of capillary dilatation and accumulation of some material-often showing stratification, along with mesangial proliferation and mesangial interposition. The accumulated material was stained positive for beta-lipoprotein by the indirect immunoperoxidase technique. Capillary dilatation resulted from gradual accumulation of the lipoprotein as well as destruction of the mesangium, as if being torn off, due to increased intraluminal pressure. The patient's eldest sister also suffers from the same renal disease, suggesting a hereditary predisposition.