A case of plane xanthoma arising in type IV hyperlipoproteinemia in a 53-year old woman is reported. The patient had well-defined yellowish to orange colored slightly elevated plaques on both upper eyelids, cheeks, neck, and upper chest. The laboratory findings showed increased serum triglyceride on lipid profile, and widening of pre-beta band on electrophoresis lipoprotein. She had a fatty liver, mild hepatomegaly, but other systemic diseases such as multiple myeloma, leukemia and lymphoma were not found.