Intrahepatic cholangiocarcinoma (ICC) is a primary liver tumor whose incidence is increasing worldwide. The prognosis is bleak, with a 5-year overall survival rate between 5% and 15%, although those with resec-table disease have a survival rate of around 30%. Most patients present at advanced stages of the disease with limited resectability at diagnosis. The most widely used staging system is the TNM system of the American Joint Committee on Cancer, which considers tumor size and lymph node involvement as crucial factors for prognosis. Surgical resection is the only curative treatment, although only about 20% of patients are resectable at diagnosis. Recurrence is common, ranging from 50% to 70% in resected patients. There are different treatment modalities, including neoadjuvant and adjuvant chemotherapy, radiotherapy, and local interventional therapies. Systemic therapy has shown limited benefits, but some studies suggest that adjuvant chemotherapy with capecitabine may improve survival. New targeted therapies and the potential role of liver transplantation in selected cases are currently under investigation.