Intrahepatic cholangiocarcinoma (ICC) is a rare and aggressive form of biliary tract cancer. The standard treatment for resectable disease is upfront surgery followed by adjuvant capecitabine. However, only 20% of patients present with resectable disease, and many develop recurrence or metastasis after curative resection. Advanced or metastatic ICCA often requires multidisciplinary care, including cytotoxic chemotherapy, targeted therapy, and locoregional therapies. Gemcitabine plus cisplatin is the first-line therapy for advanced or metastatic ICCA. Recent efforts have focused on developing more effective targeted therapies, particularly FGFR and IDH inhibitors. Despite these advancements, ICCA still has a poor prognosis. This review focuses on the current clinical management of ICCA, emphasizing surgical techniques and systemic therapies. Key topics include the importance of optimal surgical technique, guideline criteria for upfront resection, anatomic versus non-anatomic resection, margin status, lymphadenectomy, and the treatment of multifocal disease. Systemic therapies discussed include cytotoxic chemotherapy, targeted therapy (FGFR and IDH inhibitors), and immunotherapy. The article highlights the ongoing challenges and future directions in the management of ICCA.Intrahepatic cholangiocarcinoma (ICC) is a rare and aggressive form of biliary tract cancer. The standard treatment for resectable disease is upfront surgery followed by adjuvant capecitabine. However, only 20% of patients present with resectable disease, and many develop recurrence or metastasis after curative resection. Advanced or metastatic ICCA often requires multidisciplinary care, including cytotoxic chemotherapy, targeted therapy, and locoregional therapies. Gemcitabine plus cisplatin is the first-line therapy for advanced or metastatic ICCA. Recent efforts have focused on developing more effective targeted therapies, particularly FGFR and IDH inhibitors. Despite these advancements, ICCA still has a poor prognosis. This review focuses on the current clinical management of ICCA, emphasizing surgical techniques and systemic therapies. Key topics include the importance of optimal surgical technique, guideline criteria for upfront resection, anatomic versus non-anatomic resection, margin status, lymphadenectomy, and the treatment of multifocal disease. Systemic therapies discussed include cytotoxic chemotherapy, targeted therapy (FGFR and IDH inhibitors), and immunotherapy. The article highlights the ongoing challenges and future directions in the management of ICCA.