Dermal deposition of cholesterol esters was found in cutaneous papules or clinically normal skin in four of the five known living patients with Tangier disease (familial α- or high-density lipoprotein deficiency). Five patients with Niemann-Pick disease and three normal patients studied in like manner exhibited no evidence of cutaneous cholesterol ester deposition. These cutaneous findings will be useful in the diagnosis, follow-up, and study of this rare and interesting syndrome.