Muir-Torre syndrome (MTS) is an autosomal dominant genodermatosis that is characterized by the presence of at least one sebaceous gland tumor with or without keratoacanthoma, and a minimum of one internal malignancy. The most commonly associated neoplasms were colorectal (51%) and genitourinary (25%). MTS shares a number of clinical and pathological presentations with hereditary nonpolyposis colorectal carcinoma (HNPCC), including: tendency to develop right-sided colon cancers; predisposition for the development of extracolonic tumors; and prolonged survival after surgical treatment. These family members should be monitored to detect cancers preliminaly and enrolled in life-long surveillance.