ARTICLE INFO ABSTRACT Perihilar cholangiocarcinoma is one of the most challenging diseases and with no obvious symptoms at early stage, so it is difficult for surgeons to reach negative margin during surgery. Although the diagnostic accuracy of both computed tomography and magnetic resonance imaging have improved, histopathology can give the definitive diagnosis. And the recommended treatment is resection-based-multidisciplinary treatment. Now we present a case of perihilar cholangiocarcinoma.