Metastatic biliary tract cancer, a challenging disease characterized by the spread of cancer cells from the bile ducts to other parts of the body, has long been associated with a poor prognosis. However, a recent surge in research and development activities offers hope for patients. Currently, 75 drugs are in development for this cancer, with the majority in Phase II clinical trials. These drugs target various mechanisms, including programmed cell death protein 1 (PD1), vascular endothelial growth factor receptor 2 (VEGFR2), and the ERBB2 receptor. While treatment options remain limited, the pipeline of potential drugs represents a significant advancement in the fight against this devastating disease.