Cholesterol ester storage disease is a rare, inherited metabolic disorder of lipid associated with acid cholesteryl ester hydrolase deficiency. Thus far, 15 cases have been reported in the world literature. Reported here is the autopsy study of the oldest patient with this disease. The lipid storage occurred in the forms of birefringent needle-shaped crystals limited to hepatocytes and non-birefringent autofluorescent granules accumulated within the foam cells of the hepatic portal triads, duodenum, and ovaries. The cholesterol content of the liver was 16 times normal, primarily caused by increased cholesterol ester. Only trace cholesteryl ester hydrolase activity was demonstrated in the liver. An additional unique finding in our case was the presence of mesenteric lipodystrophy. Whether these two rare disorders observed in our patient represent unrelated conditions or have an etiologic association remains unknown.