It is well known that MLH1 promoter methylation, together with the presence of a BRAF V600E somatic variant and a germline pathogenic MLH1 variant, is generally considered to be a mutually exclusive mechanism in CRC carcinogenesis and that the epigenetic event is the principal mechanism of MLH1 silencing in sporadic carcinogenesis [4,7,8]. Here, MLH1 is linked to colorectal carcinoma.