A 41-year-old man had onset of disfiguring, yellow- to-orange-colored plaque skin lesions under the arms, on the axillae and scalp, and around the eyes. The lesions were largely asymptomatic. Many new lesions were beginning to develop over the face, temples and cheeks, jaw line, neck area, chest, upper back, arms, and upper and lower eyelids bilaterally. There was no internal organ involvement. His lipid profile results showed that he had a high cholesterol level (total cholesterol level, 259 mg/dL; low-density lipoprotein level, 204 mg/dL; high-density lipoprotein level, 32 mg/dL; triglycerides level, 116 mg/dL; to convert total cholesterol, low- and high-density lipoproteins, and triglycerides to millimoles per liter, multiply by 0.0259). Pathologic findings of a skin biopsy were consistent with XD.