Orthotopic liver transplantation (OLT) has many roles in biliary disease. OLT provides excellent results for patients with unresectable hilar cholangiocarcinoma. OLT prolongs survival in primary biliary cirrhosis not responsive to therapy and improves quality of life. OLT remains the durable option for patients with primary sclerosing cholangitis and complications of end-stage liver disease or recurrent cholangitis secondary to biliary obstruction. Indications for OLT after bile duct injury are chronic liver disease secondary to biliary cirrhosis and acute liver failure from associated vascular injury. OLT is treatment of choice for Caroli disease and syndrome when fibrosis leads to portal hypertension and esophageal varices.