Summary: Primary biliary cholangitis, primary sclerosing cholangitis or IgG4-dependent cholangiopathy are rare diseases of the biliary tract. Their clinical manifestation varies from asymptomatic to advanced cirrhosis with its complications. These diseases are autoimmune of unknown etiology. Beneficial for the patient is the diagnosis of the disease at an early stage, also asymptomatic, which gives a chance for optimal therapeutic management and supervision of complications. Secondary sclerosing cholangitis or ductopenia are complications of other diseases or treatment.