Here, we summarize completed, ongoing, and recruiting clinical trials in pancreatic cancer targeting CAIX-related molecular pathways, while still relying on treatment “backbones” most frequently consisting of gemcitabine, nab-paclitaxel, or the combination chemotherapy regimen FOLFIRINOX (oxaliplatin, irinotecan, fluorouracil, and leucovorin) now considered the standard first-line treatment for patients diagnosed with advanced stage pancreatic cancer, as it provides a 4.3 month increase in OS when compared to gemcitabine alone [162]. This evidence concerns the gene CA9 and pancreatic neoplasm.