Abstract: Objective To study the clinical and imaging features of hepatolith accompanying biliary duct cancer before operation, and the application of combined CA199 and imaging in its diagnosis. Methods Clinical, imaging and laboratory tumor markers examination data about 28 patients with hepatolith accompanying biliary duct cancer were retrospectively analyzed. The preoperative features of hepatolith accompanying biliary duct cancer were summarized. Results Hepatolith accompanying biliary duct cancer was diagnosed in 16 patients before operation, which was established in 10 and 2 patients by intra-operative frozen and post-operative pathologic findings, respectively. The diagnosis rate of B ultrasound, CT and MRI was 32.1%（9/28）, 53.8%（14/26） and 66.7%（8/12）, respectively. The serum CA199 level was significantly higher in patients with hepatolitn accompanying biliary duct cancer than in those with bile duct stones（P＜0.05）. No significant difference was observed in CA125 value between the two groups（P＞0.05）. Conclusion Most patients with hepatolith accompanying biliary duct cancer have a longer history of cholelithiasis, and recurrent symptoms. CT and MRI are advantageous over B ultrasound in diagnosis of biliary duct cancer. Intrao-perative frozen pathology should be performed for those if their serum CA199 level is over 500U/ml.