The cholangiopathies represent diseases and syndromes affecting the biliary system at any site between the canals of Hering and the ampulla of Vater. Hepato-canalicular cholestasis reflects biliary secretory failure of the hepatocyte caused by disturbances of intracellular organelles or damage to the bile canalicular excretory functions. Drug reactions are related especially to antibiotics, phenothiazine derivates and carbamazepine. Immune-mediated cholangiopathies cause destruction and reduction of interlobular bile ducts, and are sometimes called vanishing bile duct diseases. They include primary biliary cirrhosis, primary sclerosing cholangitis, autoimmune cholangitis, chronic hepatic allograft rejection, graft-versus-host disease and chronic cholestatic sarcoidosis. Ischemic (vascular) cholangiopathies include traumatic, hepatic arteritis and mechanical causes. Infectious cholangiopathies usually are associated with the immunosuppressed patient.