Biliary papillomatosis is a rare disease characterized by proliferation of epithelial cells in the intra- and extrahepatic biliary ducts leading to the formation of papillary outgrowths that interfere with the  bile outflow by filling up the duct lumen. Since 2010, this condition has been included in the classification of the World Health Organization as a pre-cancer pathology creating the high risk of the development of papillary cholangiocarinoma. The important role in pathogenesis of this disease is played by peribiliary glands that serve as the sources of stem cells for the replenishment of biliary epithelium. These stem cells are multipotent and may undergo differentiation into the cells of biliary, intestinal or gastric type and even into hepatocytes and  pancreatic acinar cells. A brief review of the relevant literature is presented and the problems related to  histological differential diagnostics of biliary papillomatosis based on the results of liver puncture biopsy are considered.