Abstract: Intrahepatic cholangiocarcinoma (ICC) is the second most common primary liver cancer with an incidence only secondary to hepatocellular carcinoma (HCC). The incidence of ICC in the past two decades has been significantly increased globally. Liver resection is the only well-established treatment for the disease that may achieve long-term survival, but the resectability of ICC is low and only a minority of patients may have a chance to receive radical resection. ICC usually presents with the pathological features of aggressive invasiveness such as multifocal lesions and intrahepatic infiltration, having high probability of lymph node metastasis and vascular invasion. Therefore, the long-term survival after liver resection is still suboptimal. The role of liver transplantation in the treatment of ICC remains controversial. More evidence is warranted in the effectiveness of other local and systemic therapies, as well as the targeted molecular therapy, for the treatment of advanced and intermediate ICCs that are unresectable. In recent years, immunotherapy mainly represented by checkpoint inhibitors, may provide new insights for the treatment of this disease. Authors hereby provided an updated review of ICC epidemiology, staging systems, surgical treatment, systemic chemotherapy, and immunotherapy, with emphasis on the surgical treatment and prognostic factors for ICC.