A few germline mutations related to HCC carcinogenesis have been reported recently.[8–11] TERT promoter mutation might be involved in inherited HCC,[9] and DICER1 mutation may be associated with familial recurrent liver tumors.[10] Loss of germline PKM2 promoted HCC development.[11] The most well-studied germline variations in cancer include the mutations of mismatch repair (MMR) genes, typically MLH1, MSH2, MSH6 and PMS2. The gene discussed is MLH1; the disease is hepatocellular carcinoma.