Intrahepatic cholangiocarcinoma (ICCA) is a rare and aggressive liver tumor arising from bile ducts. Surgical resection with adjuvant capecitabine is the standard treatment for resectable cases, but only 20% of patients present with resectable disease. Many develop recurrence or metastasis after surgery. Advanced or metastatic ICCA requires multidisciplinary care with chemotherapy, targeted therapy, and locoregional treatments. Gemcitabine plus cisplatin is the first-line therapy for advanced ICCA. Recent efforts focus on FGFR and IDH inhibitors for targeted therapy, though ICCA still has a poor prognosis. Surgical techniques for ICCA include anatomic and non-anatomic resections. Anatomic resection is preferred for tumors in a single segment, while non-anatomic is used for small, peripheral lesions. Achieving an R0 margin is crucial for curative intent. Lymphadenectomy is essential for staging and prognosis. Patients with multifocal ICCA may benefit from resection, but the optimal approach remains unclear. Hepatic artery infusion pump (HAIP) delivers chemotherapy directly to the liver, improving survival in some cases. Systemic chemotherapy, such as gemcitabine plus cisplatin, is used for advanced ICCA. Abraxane (nab-paclitaxel) may enhance gemcitabine delivery by reducing stromal tissue. Targeted therapies, including FGFR and IDH inhibitors, show promise. FGFR inhibitors like infigratinib and futibatinib have shown efficacy in clinical trials. IDH inhibitors like ivosidenib target mutations in IDH1 and IDH2, improving survival in patients with IDH-mutated ICCA. Immunotherapy, such as durvalumab and pembrolizumab, has shown some benefit in advanced ICCA. However, long-term outcomes remain challenging. Future directions include developing new systemic therapies and improving surgical techniques to enhance survival and outcomes for ICCA patients.Intrahepatic cholangiocarcinoma (ICCA) is a rare and aggressive liver tumor arising from bile ducts. Surgical resection with adjuvant capecitabine is the standard treatment for resectable cases, but only 20% of patients present with resectable disease. Many develop recurrence or metastasis after surgery. Advanced or metastatic ICCA requires multidisciplinary care with chemotherapy, targeted therapy, and locoregional treatments. Gemcitabine plus cisplatin is the first-line therapy for advanced ICCA. Recent efforts focus on FGFR and IDH inhibitors for targeted therapy, though ICCA still has a poor prognosis. Surgical techniques for ICCA include anatomic and non-anatomic resections. Anatomic resection is preferred for tumors in a single segment, while non-anatomic is used for small, peripheral lesions. Achieving an R0 margin is crucial for curative intent. Lymphadenectomy is essential for staging and prognosis. Patients with multifocal ICCA may benefit from resection, but the optimal approach remains unclear. Hepatic artery infusion pump (HAIP) delivers chemotherapy directly to the liver, improving survival in some cases. Systemic chemotherapy, such as gemcitabine plus cisplatin, is used for advanced ICCA. Abraxane (nab-paclitaxel) may enhance gemcitabine delivery by reducing stromal tissue. Targeted therapies, including FGFR and IDH inhibitors, show promise. FGFR inhibitors like infigratinib and futibatinib have shown efficacy in clinical trials. IDH inhibitors like ivosidenib target mutations in IDH1 and IDH2, improving survival in patients with IDH-mutated ICCA. Immunotherapy, such as durvalumab and pembrolizumab, has shown some benefit in advanced ICCA. However, long-term outcomes remain challenging. Future directions include developing new systemic therapies and improving surgical techniques to enhance survival and outcomes for ICCA patients.