The occurrence of cutaneous xanthomatous lesions and hyperlipemia in the presence of hepatic dysfunction has only recently been recognized as a single clinical entity. First in 1937 Thannhauser and Magendantz,10 and later in 1940 Thannhauser11 described and correlated this combination and called it "xanthomatous biliary cirrhosis." Since that time there have been only about 30 cases with autopsy reported in the medical literature. It is the purpose of this paper to report one additional necropsied case, and to include a very brief review of the clinical and pathologic picture of this disease. The patient was a 36 year