Muir-Torre syndrome is a rare autosomal dominant disorder characterized by unusual sebaceous neoplasms and visceral malignancy. Excluding sebaceous hyperplasia and sebaceous nevus of Jadassohn, sebaceous neoplasms occur so rarely that the presence of a sebaceous neoplasm mandates consideration of Muir-Torre syndrome. Sebaceous neoplasms precede or are synchronous with visceral malignancies in up to 40% or more of patients with Muir-Torre syndrome, and the presence of such a lesion warrants an evaluation for visceral malignancy.