Muir-Torre syndrome (MTS) is a rare genodermatosis characterized by the occurrence of sebaceous gland neoplasms and/or keratoacanthomas associated with visceral malignancies. It is autosomal dominantly inherited and considered as a subtype of Lynch 2 syndrome, which is a hereditary nonpolyposis colorectal cancer syndrome. Sebaceous adenoma, sebaceoma, sebaceous carcinoma and keratoacanthoma are the most frequent associated cutaneous neoplasms. The most common visceral malignancies are colorectal and genitourinary tumors. A 47-year-old male patient was admitted to our clinic with the complaint of two lesions on the nose. Dermatological examination revealed a plaque 1 cm in diameter consisting of bright yellowish-white colored papules with slightly umbilicated appearance, and telangiectasias on the left side of the nose and a dome-shaped papule 3 mm in diameter with hyperkeratotic plug on the tip of the nose. He had a history of partial colon resection due to colon cancer and familial Lynch II syndrome. On dermoscopic examination of sebaceous adenoma, a few yellow comedo-like globules and branching arborizing vessels were detected. Reflectance confocal microscopy (RCM) revealed a good histopathologic correlation. Sebaceous lobules were composed by clusters of ovoid cells with hyporefractile dark nuclei and bright, hyperrefractile glistening cytoplasm. Numerous round to oval-shaped dark spaces corresponding to sebaceous ducts were detected. The diagnosis of MTS was established based on the personal and family history, dermoscopic, RCM and histopathologic findings. MTS evaluation is required in patients with biopsy-proven sebaceous adenoma. Early diagnosis may be lifesaving in patients with MTS. A better characterization of RCM features of sebaceous tumors will allow early diagnosis of the patients with MTS.