Currently, pemigatinib, which inhibits Fibroblast Growth Factor Receptor 1 (FGFR1), FGFR2 and FGFR3, as well as ivosidenib, which targets mutant isocitrate dehydrogenase-1 (IDH1), remain the only targeted agents approved by the European Medicines Agency for the treatment of CCA following the promising findings of the Phase II FIGHT-202 and ClarIDHy trials [1,5,6]. The gene discussed is IDH1; the disease is cholangiocarcinoma.