This paper reviews cholestatic liver diseases which are characterized by disappearance of the intrahepatic bile ducts ("vanishing bile duct" diseases). In neonates and children, the most important entities are extrahepatic bile duct atresia and paucity of intrahepatic bile ducts, including syndromatic and nonsyndromatic varieties. Immunological mechanisms play a role in the bile duct obstruction observed in primary biliary cirrhosis, graft-versus-host disease and chronic liver transplant rejection, and possibly also in primary sclerosing cholangitis and sarcoidosis with chronic intrahepatic cholestasis. Idiopathic adulthood ductopenia represents a newly defined entity, the nature of which remains unclear. Mention is made of recently reported forms of intrahepatic bile duct destruction due to toxins and drugs (iatrogenic cholangiopathies). New insight into the pathogenesis of these diseases has been brought about by progress in immunology, imaging techniques of the liver and hepatobiliary surgery.