We present the case of a 44-year-old male patient with a history of primary sclerosing cholangitis who developed a cholangiocarcinoma with pulmonary metastasis. The cholangiocarcinoma was identified in the PET scan by its enhanced 18F-FDG-uptake. Prospective studies should be performed to demonstrate whether PET will be suited to detect small and early cholangiocarcinomas, particular in young patients, who could be treated immediately and curatively by liver transplantation.