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Background: The management of hepatolithiasis combined with intrahepatic cholangicarcinoma (IHHCC) remains a challenge due to poor prognosis. The aim of this study was to summarize our diagnosis and cure experience of IHHCC over the recent 10 years. Methods: From January 1996 to January 2006, 66 patients with IHHCC were reviewed retrospectively. Results: Of the 66 patients, 52 underwent surgical resection (radical resection in 38 and palliative in 14) and 8 patients abdominal exploration, while the other 6 cases received endoscopic retrograde biliary internal drainage and stent implantation. In this series, correct diagnosis of advanced stage was made during operation in 8 cases (8/60, 13.3%) and all of them (underwent unnecessary abdominal exploration, among them the positive rate of CA19-9 was 100%, and the positive rate of CEA was 87.6% (7/8), incidence rate of ascites was 100% and short-term significant weight loss was 100%, with median overall survival of only 4 months. Conclusion: Radical resection is mandatory for IHHCC patient to achieve long-term survival, the CT and MR imaging features of IHHCC being concentric enhancement. Patients with IHHCC have significant higher CA199 and significant higher CEA and short-term significant weight loss and ascites should be considered with advanced stage of IHHCC and unnecessary non-therapeutic laparotomies should be avoided.