Gamma-glutamyltransferase (GGT) is a microsomal enzyme that is widely distributed in human tissues involved in secretory and absorptive processes, particularly the bile canaliculi. Serum GGT is elevated in liver diseases affecting the biliary system, such as extrahepatic biliary atresia, sclerosing cholangitis and progressive familial intrahepatic cholestasis (PFIC) type 3. Conversely, two other subtypes of PFIC have normal or low serum GGT activity, discordant with the degree of cholestasis. GGT is also useful in detecting cholestasis associated with parenteral nutrition and assessing the efficacy of ursodeoxycholic acid in its treatment. GGT may also help screening for biliary complications on patients that have undergone orthotopic liver transplantation. The reference range for GGT is age dependent. In normal full-term neonates the activity at birth is approximately six to seven times the upper limit of the adult reference range. The activity then declines, reaching adult levels by the age of 5-7 months.