Here, we describe a family with several affected members who have a novel homozygous variant in FAH (Chr15(GRCh38):g.80162305A > G; NM_000137.2:c.424A > G; NP_000128.1:p.R142G) with undetectable SUAC and normal amino acid levels but extensive cirrhosis and susceptibility to hepatocellular carcinoma (HCC). Here, FAH is linked to hepatocellular carcinoma.