Abstract
A 62 year old housewife had marked increase and accumulation of glycolipids in the liver and the spleen . Comparison of the clinical findings, patho-anatomical findings and results of chemical analysis revealed resemblance to the changes in the skin, smooth muscle, heart and kidney, in this case, to those of Fabry's disease, while the ocular and reticulo-endothelial findings resembled Gaucher's disease . However the entire picture was different from Fabry's and Gaucher's disease . From this autopsy case, relationship between smooth muscle and glycolipids was mainly discussed.
