The dysfunction of ABCB11 leads to cholestatic liver diseases, the most severe form being progressive familial intrahepatic cholestasis type 2 (PFIC2), which is a rare autosomal recessive disease affecting patients harboring a homozygous or a compound heterozygous status for ABCB11 variations early during childhood [1,3]. Here, ABCB11 is linked to Cholestatic liver disease.