Xanthomas are localized lipid deposits within organs that may manifest as papules, plaques, or nodules in skin. They are commonly associated with all types of hyperlipidemia. Xanthoma striatum palmare characterized by xanthomas of the palmar creases is a rare but important diagnostic physical sign of dysbetalipoproteinemia, also known as type III hyperlipoproteinemia. Type III hyperlipoproteinemia is characterized by the early onset of cardiovascular disease and peripheral vascular disease. We describe herein the case of a 51-year-old female patient affected by xanthoma striatum palmare associated with elevated plasma levels of triglycerides and cholesterol and a lipoprotein electrophoresis pattern consistent with type III hyperlipoproteinemia. (Korean J Dermatol 2018;56(3):187∼191)