A 43 year-old Korean male has had multiple, grouped, asymptomatic, yellowish brown flat papules and nodules on the knees, elbows, buttocks, palms and soles for 3 years. Small yeIlowish, discrete papules first appeared on the right palm and left elbow, and gradually increased in size and number and spread to knees, buttocks and soIes. Physical examination revealed normal except for the skin lesions and no familial occurrence was noted. Gross finding of the serum was turbid, more or less creamy and paper electrophoresis revealed marked increase of prebetalipoprotein, increase of betalipoprotein and alphalipoprotein and absence of chylomicron, suggesting type III or type IV hyperlipoproteinemia. Serem choleterol was 200mg% and fasting blood sugar was 115.0mg%. with normal glucose tolerance test. Histopathology showed many aggregates of foam cells on H-E stain and many lipid droplets in the dermis on Sudan red stain. Based on the clinical and histopathological findings and lipoprotein analysis by the paper electrophoresis, the patient was diagnosed as type IV hyperlipoproteinemia with xanthoma tuberosum.