Lynch syndrome is an autosomal dominant disorder caused by germline mutation affecting mismatch-repair genes. Genetic testing is performed selectively. Diagnosed individuals are to undergo surveillance to detect and manage Lynch syndrome-related cancers early in the course. Muir-Torre syndrome is a phenotypic variant of Lynch syndrome characterized by sebaceous neoplasms, keratoacanthoma, or both in addition to other Lynch syndrome-related cancers. Other neoplasms of the skin, such as squamous cell carcinoma, are not recognized as part of the Lynch syndrome tumor-spectrum. We report a case of cutaneous squamous cell carcinoma occurring in a patient with Lynch syndrome and explore some of the characteristic features and significance of this association.