AbstractIntrahepatic biliary papillomatosis is most often the initial stage of diffuse biliary papillomatosis. Without any specific clinical expression, the disease may mimic the symptomatology of biliary lithiasis. It is usually discovered either during surgery or postoperatively by the presence of mucus discharge via the drainage system of the biliary tract. Its course is unfavorable because of its tendency to extend to the entire biliary tree and even to the pancreatic ductal system. Papillomatosis may degenerate, as recent publications have shown. Numerous therapeutic approaches have been proposed, including transcanalicular extraction, drainage, and hepatic excision. Most often, these measures are only palliative because of the unrelenting extensive character of the disease. Prognosis is bleak. Perhaps some hope may be provided by hepatic transplantation.