Cholangiocarcinoma is a malignant neoplasm that affects the epithelium of the bile duct. It has low incidence and prevalence, usually appearing after the sixth decade of life, with a slight predominance in the male population. It mostly affects the confluence of the hepatic ducts, where it is called "hilar cholangiocarcinoma" or "Klatskin tumor". Its etiology is still partially unknown, but it has well-defined predisposing conditions and risk factors such as primary sclerosing cholangitis, parasitic infections, and exogenous toxins. The initial clinical presentation is nonspecific and can later include jaundice, weight loss, and right upper abdominal pain. Diagnosis is confirmed through clinical alterations, laboratory tests, imaging, and histopathology. Two clinical staging classifications are used: Bismuth-Corlette and TNM. The gold standard treatment is surgical excision with negative histological margins. However, the resectability rate is still low due to late diagnosis. Patients with unresectable tumors may benefit from palliative treatment, such as endoscopic stent placement or percutaneous drainage. New chemotherapy/phototherapy treatments are being studied to improve the quality of life of patients.