ABSTRACTXanthomata are soft tissue tumors composed of lipid-laden “foamy” histiocytes associated with cholesterol clefts and inflammation. They are considered to be specialized granuloma rather than true neoplasmas and are usually associated with disorders of lipid metabolism, most commonly one of the hyperlipoproteinemia syndrome. A rare case of a xanthoma involving the temporal bone and causing extensive destruction of the skull base is presented. Otalgia and infection are the most common presenting symptoms of this disorder, with multiple cranial nerve palsies resulting in some cases. Treatment includes conservative surgical debulking, dietary restriction of fat and cholesterol, and pharmacologic reduction of serum lipids.