The MLH1 c.400A > G p.Lys134Glu VUS identified, occurred in a tumor with loss of MLH1/PMS2 expression and with two somatic MLH1 mutations, where one of these may function as the “second hit” on the wildtype allele, however, further characterization of this variant is needed to determine whether this person has Lynch syndrome or double somatic MMR mutation-related dMMR. The gene discussed is MLH1; the disease is Lynch syndrome.