Muir-Torre syndrome (MTS) is an autosomaldominant skin condition of genetic origin, characterized by tumors of the sebaceous glands or keratoacanthomas that are associated with malignant visceral diseases. MTS associated with gynecologic malignancy has rarely been reported. Here we report a woman with no family history of colorectal cancer who developed endometrial carcinoma, stage 3a, at 49 years of age and at age 51 years, developed two skin tumors, a nasal squamous cell carcinoma and a sebaceous carcinoma of the right eyelid. The appearance pattern of these skin tumors suggested MTS. Although MTS associated with endometrial carcinoma is rare, patients with endometrial carcinoma should undergo evaluation for visceral malignancies (mainly colon cancer) and sebaceous skin lesions, regardless of whether or not there is a family history of colorectal cancer.