The Muir-Torre syndrome (MTS) is an autosomal dominant genodermatosis characterized by: (1) at least a single sebaceous gland tumor (either an adenoma, an epithelioma, or a carcinoma) and (2) a minimum of one internal malignancy. To date, 120 patients with MTS have been reported. The most commonly associated neoplasms were colorectal (51%) and genitourinary (25%). Unlike colorectal neoplasms in the general population, the majority (58%) of these tumors in MTS patients occurred proximal to or at the splenic flexure. Nearly half of the MTS patients had more than one primary malignancy. Cutaneous lesions occurred before or concurrent with the diagnosis of the initial cancer in 41% of these patients. The median age for the appearance of the skin lesions was 53 years (range, 23 to 89 years); the median age for the detection of the initial visceral neoplasm was 50 years (range, 23 to 81 years). The cancers appear to have an indolent course in many of the MTS patients; the median survival has not been reached and the median follow-up is 10+ years. Patients with an MTS-associated cutaneous lesion should have a complete evaluation for gastrointestinal or genitourinary cancers. Although the penetrance of this disease is variable, its autosomal dominant inheritance suggests that relatives should be examined for sebaceous gland tumors and internal malignancy.