Intrahepatic cholangiocarcinoma (IHCC) is a primary tumor originating in the bile ducts, classified into intrahepatic, perihilar (Klatskin tumor), and distal types, each presenting different epidemiology, biology, and prognosis. It is challenging to manage, with a grim prognosis due to late diagnosis. IHCC represents 10-20% of primary liver cancers, with a global annual incidence of 0.3-6 per 100,000 inhabitants and significant geographical variations, being more prevalent in Southeast Asia. An increase in incidence has been observed over the past 2 to 3 decades. It affects more men and is rare in individuals under 40 years old, except in cases of Primary Sclerosing Cholangitis (PSC). The main risk factors are related to chronic inflammation of the bile ducts and include PSC, primary biliary lithiasis, congenital cysts of the biliary system, and hepatitis B and C infections, among others. IHCC is generally an adenocarcinoma with different growth patterns and histology. In the early stages, it is usually asymptomatic. The most common symptoms in advanced stages are abdominal pain, weight loss, and hepatomegaly. Ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and PET-CT are used for diagnosis, with CT and MRI preferred to assess resectability and lymph node involvement. Tumor markers such as CA 19-9 and CEA aid in diagnostic support, although with low sensitivity.