The Muir-Torre syndrome is a rare disorder characterized by sebaceous neoplasms of the skin and multiple visceral malignancies. The syndrome appears to be a familial, autosomal dominant condition. We diagnosed this syndrome in a previously unreported patient and found a personal and family history of malignancies and hyperlipidemia. The association of Muir-Torre syndrome with a family history of hyperlipidemia, another autosomal dominant condition, has not been previously reported. The possible genetic relationship between the two disorders is discussed.