Muir-Torre syndrome is defined by concurrent or sequential development of internal malignancy and sebaceous neoplasm or multiple keratoacanthomas. Muir-Torre syndrome is very rare, with only 205 cases reported in the literature. We reported a patient with Muir-Torre syndrome with three internal malignancies. A 64-year-old patient with a history of breast cancer, stomach cancer and colon cancer visited our department for treatment of the skin lesion that occurred five years before on the left cheek. The lesion was excised completely with a resection margin of 1 cm, followed by full-thickness skin graft from left postauricular area for reconstruction. Histopathology revealed a 0.2 × 0.2 × 0.1 cm sized sebaceous carcinoma with 4 mm safety margin. The skin graft was well taken within 7 days after surgery and the patient was discharged to outpatient follow-up. There was no complication related with surgery. Muir-Torre syndrome is very rare, as are sebaceous gland tumors. So if a cancer of the sebaceous gland is diagnosed, screening workup for internal malignancy is recommended. Because of its good prognosis, surgical removal of primary or metastatic cancers may be curative and should be attempted where possible.