Abstract: Intrahepatic cholangiocarcinoma (ICC) is a group of adenocarcinoma that derives from epithelium of the subbranches of intrahepatic bile ducts, which is characterized by difficulty in early diagnosis, high malignancy and poor prognosis. ICC is relatively rare, but it is the second most common in primary liver cancer, and its incidence has gradually increased worldwide. Radical resection is recognized as the only treatment that can bring better long-term survival currently. However, many surgery-related problems, such as lymph node dissection and major vascular resection and reconstruction, are still controversial. Adjuvant therapy, including chemotherapy, regional therapy, targeted therapy and immunotherapy, has received increasing attention in recent years. Although there is no uniform standard, a large number of related studies have supported its efficacy. In this article, the authors have reviewed the molecular pathogenesis of ICC and advances in treatment.