The Muir-Torre syndrome is an autosomal dominantly inherited genodermatosis with malignant potential that is characterized by the presence of at least one sebaceous gland tumor (adenoma, epithelioma, or carcinoma) and a minimum of one internal malignancy. The syndrome has been documented in 147 individuals. Associated features in some of the Muir-Torre syndrome patients are colorectal carcinomas and genitourinary neoplasms. More than half of the 292 visceral cancers described in Muir-Torre syndrome patients were colorectal carcinomas; nearly 60% of these tumors were located at or proximal to the splenic flexure. The presence of even a single Muir-Torre syndrome-associated sebaceous tumor warrants serious consideration for further evaluation of that individual for the syndrome. Therefore, initial and periodic examination for internal malignancy should be performed in individuals with such tumors and patients with the syndrome. Also, family members of Muir-Torre syndrome patients should be screened for Muir-Torre syndrome-associated cutaneous lesions and visceral cancers.