These variant syndromes include Muir-Torre syndrome (autosomal dominant) due to MSH2 and MLH1 genes mutations and characterized by the presence of cutaneous manifestations (multiple sebaceous adenomas, epithelioma, and keratoacanthoma) associated with colorectal and endometrial cancers and Turcot syndrome (autosomal dominant) associated with APC, PMS2, and MLH1 genes mutations and characterized by brain cancers (glioblastoma and cerebellar medulloblastoma) are associated with colorectal cancer [6, 7]. Here, MLH1 is linked to keratoacanthoma.