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It has been confirmed that many risk factors are associated with ICC including intrahepatic lithiasis, primary sclerosing cholangitis (PSC), congenital abnormalities of the bile ducts, parasite infection, toxic exposures chronic liver disease (viral infection and cirrhosis) and metabolic abnormalities. In recent years, significant progress has been made in the clinical diagnosis and treatment of ICC. Advances in functional and molecular imaging techniques offer the possibility for more accurate preoperative assessment and detection of recurrence. Moreover, the combination of molecular typing and traditional clinical pathological typing provides accurate guarantee for clinical decision-making. Surgical resection is still the only radical treatment for ICC, while R0 resection, lymph node dissection, postoperative adjuvant therapy and recurrence resectomy have been confirmed to be beneficial for patients. 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