Abstract: Nowadays, the global occurrence of biliary tract carcinoma is on the rise. The current systemic treatment is mainly chemotherapy, but the clinical efficacy is extremely limited, mainly due to its high degree of heterogeneity at the genomic, epigenetic and molecular levels. The emergence of targeting therapy and immunotherapy has opened up a new channel for the treatment of biliary tract carcinoma. In the past years, in-depth exploration of the complexity of biliary tract carcinoma has been conducted and new therapies that may help improve the prognosis of patients have been developed. This paper reviews the latest research progress in the field of biliary tract carcinoma, in the hope of providing further guidance for clinical practice.