Intriguingly, approximately 40–50% of patients with iCCA harbor actionable mutations and alterations, including fibroblast growth factor receptor (FGFR) fusions/rearrangements (10–16%), isocitrate dehydrogenase-1 (IDH1) mutations (10–20%), and BRAF mutations (<5%); in addition, neurotrophic tyrosine receptor kinase (NTRK) gene fusions (<5%) and high microsatellite instability (MSI-H)/tumor mutational burden high (TMB-H; <5%) have been identified as rare but potential targets for therapy [10,15,25,27] (Table 1). This evidence concerns the gene BRAF and infantile convulsions and choreoathetosis.