The serum levels of interleukin 6, trypsinogen, mucin-5AC, soluble fragment of cytokeratin 19 and the platelet-lymphocyte ratio have been recently shown to help in the diagnosis of CCA with, in some cases, a prognostic value. As far as bile is concerned, the ratio of pancreatic elastase/amylase, mucin-4, minichromosome maintenance replication protein and insulin-like growth factor 1 have been explored, with the insulin-like growth factor 1 biliary concentration capable of completely discriminating CCA from benign biliary disorders and pancreatic cancer. We have also discussed advances in the proteomic of serum and bile, which seem promising in identifying new markers for CCA.