A 67-year-old man complained of impaired vision at night for several months. He was known to have arcus senilis (arcus lipoides corneae) since aged 21 years. For the last 20 years both corneas had become progressively more cloudy. His mother was said to have had similar eye changes. Ophthalmological examination discovered no abnormality other than marked corneal dystrophy with hardly separable arcus senilis. General physical examination was normal. Total cholesterol and triglyceride levels in serum were within normal limits, but serum concentration of high density lipoprotein (HDL) cholesterol (8 mg/dl) was reduced as were, within the high density lipoprotein fraction, the concentrations of triglyceride (4 mg/dl), phospholipids (38 mg/dl), the proportion of cholesterol esters (31%), and the cholesterol-esterification rate (51 nmol/ml . h). The HDL-associated activity of lecithin-cholesterol-acetyltransferase activity was scarcely measurable (0.9 nmol/ml . h). The signs in this case (cloudy cornea, marked decrease in serum HDL cholesterol concentration without premature arteriosclerosis) are typical of fish eye disease.