The normality of GGT level in intrahepatic cholestasis is exceptional and tends to occur in infants or young children with some types of familial intrahepatic cholestasis, such as progressive familial intrahepatic cholestasis types 1 and 2 or in adenosine triphosphate‐binding cassette (ABCB4) mutations. This evidence concerns the gene ABCB4 and progressive familial intrahepatic cholestasis type 1.