Furthermore, fibroblast growth factor receptor 2 (FGFR2) fusions are found in 10–15% of patients with iCCA and results from the phase II FIGHT-202 study led to the recent Food and Drug Administration (FDA) and European Medicines Agency (EMA) approval of the FGFR inhibitor pemigatinib for the treatment of iCCA based on high radiological response rates in pre-treated patients with durability of the observed responses [36]. Here, FGFR2 is linked to infantile convulsions and choreoathetosis.