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AIM: The diagnosis of cholangiocarcinoma is often difficult,making management approaches problematic. A reliable serum marker for cholangiocarcinoma would be a useful diagnostic test. The aims of our study were to evaluate the usefulness of a serum CA19-9 determination in the diagnosis of cholangiocareinoma.METHODS: We prospectively measured serum CA19-9 and CEA concentrations in patients with cholangiocarcinoma (n=35), benign biliary diseases (n=92), and healthy individuals n=15). Serum CA19-9 and CEA concentrations were measured by an immunoradiometric assay without knowledge of the clinical diagnosis.were 77.14% and 68.57%, respectively. When compared with the benign biliary diseases group, the true negative rates of serum CA19-9 and CEA were 84.78% and 81.52%,respectively, The false positive rates of serum CA19-9 and CEA were 15.22% and 18.48%, whereas the accuracy of serum CA19-9 and CEA were 82.68% and 77.95%,respectively. Serum CA19-9 and CEA concentrations were significantly elevated (P＜0.001 and P＜0.05) in patients with patients undergoing curative resection of cholangiocareinoma,the mean serum CA19-9 concentration was decreased from however, no correlation was found between serum CEA and CA19-9 concentrations (r=0.036).CONCLUSION: These data suggest that the serum CA19-9 determination is a usefol addition to the available tests for the differential diagnosis of cholangiocareinoma. Serum CA19-9 is an effective tumor marker in diagnosing cholangiocareinoma,deciding whether the tumor has been radically resected and monitoring effect of treatment.