Abstract: Cholangiocarcinoma (CCA) is a category of highly heterogeneous and aggressive malignancy mainly originating from bile duct epithelial cells. The median survival time of untreated CCA patients is approximately 12‒24 months, and the effectiveness and durability of surgical resection and neoadjuvant chemotherapy are limited. Results of the next-generation sequencing show that dysregulation of the immune system plays an important role in the pathogenesis of CCA. It has opened up new possibilities for the study of therapies targeting the natural course of aggressive CCA, such as immune checkpoint inhibitors, adoptive cell therapy, and tumor vaccines. Based on the current status of immunotherapy for CCA, the authors review the efficacy and dilemmas of current CCA immunotherapy strategies and look forward to the future treatment prospects of CCA.