This manuscript reviews and summarizes the histopathology of the liver in the various "vanishing bile duct diseases". These represent conditions associated with progressive disappearance of intrahepatic bile duct branches. Congenital diseases of this nature comprise so-called extrahepatic bile duct atresia and paucity of interlobular bile ducts (syndromic and non-syndromic). In the adult, vanishig bile duct diseases are represented amongst others, by primary biliary cirrhosis, primary sclerosing cholangitis, idiopathic adulthood ductopenia, liver allograft rejection, graft-versus-host disease and drug-induced and toxic cholangitis.