Lynch syndrome (LS) which constitutes the most common form of hereditary CRC and accounts for approximately 3% of the total CRC burden [2,3], is inherited in an autosomal dominant fashion with mutations most commonly found in the mismatch repair genes (MMRs), namely MLH1, MSH2, MSH6 and PMS2 [4,5]. The gene discussed is MSH6; the disease is colorectal carcinoma.