As matter of fact then, several genes involved in the development of inherited cholestasis are linked to the risk of HCC and CCA in pediatric and not pediatric populations: ABCB11, ABCB4, TJP2, FXR, MYO5B, SLC51B, SLC25A13, NOTCH2, JAG1, TGR5 and HNF1B [5,6,7]. The gene discussed is NR1H4; the disease is cholangiocarcinoma.