As reported in the literature, LS subjects have a higher risk of developing one or more CRC with cumulative incidences by the age 75 years of 48.3–57.1% for MLH1, 46.6–51.4% for MSH2, 18.2–20.3% for MSH6 and 12.8% for PMS2 [2,3]. Here, MLH1 is linked to colorectal carcinoma.