Intrahepatic cholangiocarcinoma (iCCA) is a malignant neoplasm of the biliary tract, the incidence of which has increased in recent years. The etiopathogenesis is not fully elucidated, but the greatest association has been shown with inflammatory changes within the biliary tract. Surgical treatment is the main therapeutic modality; however, less than 30% of its are resectable at diagnosis, with the majority of patients requiring systemic treatment. Chemotherapy with capecitabine is the standard adjuvant therapy. For patients with inoperable tumors or metastatic lesions, chemotherapy alone or in combination with immunotherapy (durvalumab, pembrolizumab) is used. There is a need to provide systemic treatment in patients with progression after first-line treatment in good performance status. New therapeutic pathways for the treatment of this tumor type are still being identified with new emerging potential targets such as isocitrate dehydrogenase (IDH), fibroblast growth factor receptor 2 (FGFR2), or BRAF mutation.