Genetic testing should also be considered such as mutations in the hepatobiliary transporters ATP8B1, ABCB11 and ABCB4 in the evaluation of familial hepatocellular cholestasis like progressive familial intrahepatic cholestasis (PFIC) or benign recurrent intrahepatic cholestasis (BRIC). Here, ABCB11 is linked to progressive familial intrahepatic cholestasis.