In Western countries, pediatric liver cancers were reported to occur in patients with PFIC; notably, 5–15% of children with PFIC2 present HCC early, from 13 to 28 months of age [38,39,40], but several cases are well described in infants with TJP2 disease (PFIC4) and MDR3 deficiency (PFIC3) [41,42,43,44]. The gene discussed is TJP2; the disease is hepatocellular carcinoma.