MLH1 promoter methylation in Lynch Syndrome patients has been described before, either co-occuring with a pathogenic germline MSH6 variant in a patient with a urothelial carcinoma at age 70 [30], in a patient with a pathogenic germline MLH1 variant in a CRC at the age of 59 [31] or with pathogenic germline MSH6 variant in a patient with multiple primary cancers, from the age of 56 [32]. Here, MLH1 is linked to Lynch syndrome.