We performed searches of Pubmed/Medline, the Cochrane library, and Scopus using the keywords “biliary tract cancer” OR “intrahepatic cholangiocarcinoma” OR “extrahepatic cholangiocarcinoma” OR “perihilar cholangiocarcinoma” OR “distal cholangiocarcinoma” OR “gallbladder carcinoma” OR “ampulla of Vater” AND “antiangiogenic therapy” AND “immunotherapy” OR “immune checkpoint inhibitors” OR “PD-L1 inhibitors” OR “immunotherapy and antiangiogenic combined therapy”. The gene discussed is CD274; the disease is hilar cholangiocarcinoma.