Progressive familial intrahepatic cholestasis Type 3 (PFIC3) is a rare, autosomal recessive, and hepatocellular-originating cholestatic liver disease caused by mutations in the ABCB4 gene, which encodes the multidrug resistance protein 3 (MDR3) [1]. Here, ABCB4 is linked to progressive familial intrahepatic cholestasis type 3.