Prompted by the histomorphological aspect of ductopenia, chronic intrahepatic liver diseases are increasingly being subsumed under the term vanishing bile duct syndrome. Classification by cholestasis syndromes in adults (e.g. primary biliary cirrhosis, primary sclerosing cholangitis) and in the newborn or children (e.g. alphal antitrypsin deficiency, cystic fibrosis) makes good sense. Decisive for the diagnosis are, depending on the disease presenting, a typical constellation of laboratory results, detection of autoantibodies, imaging procedures (e.g. ERC, MRI), liver biopsy where indicated, or suspected drug-induced cholestasis. Byway of treatment, ursodeoxycholic acid, an antibiotic in cholestasis, and liver transplantation in some cases, are possible options. Supportive treatment should be aimed at extrahepatic manifestations of cholestasis (e.g. osteoporosis, vitamin deficiency, pruritus).