Cholangiocarcinoma (CCA) is a malignant tumor originating from the epithelial cells of the bile ducts, with its incidence and mortality rates rising in recent years, particularly in Asia. Because the early symptoms of CCA are often insidious and nonspecific, most patients are diagnosed at an advanced stage and lose the opportunity for radical surgical treatment. Although chemotherapy remains the main first-line therapy, its efficacy is limited. With the rapid development of next-generation sequencing technologies and the growing emphasis on individualized precision medicine, our understanding of the molecular biology of CCA has deepened substantially. Research on molecular targeted therapy, immunotherapy, and combination strategies integrating both approaches has made remarkable progress. This article provides an overview of the molecular targets, immune microenvironment, immune checkpoint inhibitors, and therapeutic strategies related to CCA.