Muir-Torre syndrome is a genodermatosis characterized by the presence of at least one sebaceous gland neoplasm and at least one visceral malignancy. This rare disorder was first described by Muir et al in 1967 and Torre in 1968. Characteristic sebaceous neoplasms include sebaceous adenoma, sebaceous carcinoma, sebaceoma, and keratoacanthoma with sebaceous differentiation. The most common visceral malignancies are colorectal and genitourinary tumors. An 69-year-old male presented with an asymptomatic 1.0x0.7 cm sized erythematous papule on right dorsum of hand, of 12 months duration. The patient had a history of retroperitoneal nephroureterectomy for right lower ureteral cancer and 3 years later, total nephrectomy for both renal cancer. And he had a history of defoliant exposure and treatment of numerous actinic keratotic lesions by aldara and cryotherapy on both arms and face for 1 year. The punch biopsy confirmed squamous cell carcinoma. He was given Mohs surgery with 2 mm margins. After 5 months of the treatment he visited clinic, with numerous erythematous papules on face, scalp and neck. Biopsies showed squamous cell carcinoma, sebaceous adenoma, keratoacanthoma, and sebaceous hyperplasia. These lesions and visceral malignancies were compatible with Muir-Torre syndrome. For further evaluation for Muir-Torre syndrome, we suggested hMSH2 and hMLH1 gene evaluation. but he refused the proposal because clinical criteria have been met.