Cholangiocarcinoma, a diverse group of epithelial tumors with cholangiocyte differentiation, is categorized into intrahepatic (iCCA), perihilar (pCCA), and distal (dCCA) subtypes. Each subtype has distinct epidemiology, biology, prognosis, and clinical management strategies. The incidence of cholangiocarcinoma, particularly iCCA, has increased globally over the past few decades. Surgical resection is the primary curative treatment for all subtypes, while liver transplantation after neoadjuvant chemoradiation is limited to early-stage pCCA. For advanced or unresectable disease, locoregional and systemic chemotherapeutics are the main treatment options. Advances in external-beam radiation therapy have improved treatment outcomes. Molecular profiling has identified promising targets for precision medicine, including immunotherapy. Biomarker-driven trials, stratifying patients by subtype and genetic aberrations, are essential for developing targeted therapies. Targeting the rich tumor stroma in conjunction with targeted therapies may also be beneficial. The article reviews the evolving developments in the epidemiology, pathogenesis, and management of cholangiocarcinoma.Cholangiocarcinoma, a diverse group of epithelial tumors with cholangiocyte differentiation, is categorized into intrahepatic (iCCA), perihilar (pCCA), and distal (dCCA) subtypes. Each subtype has distinct epidemiology, biology, prognosis, and clinical management strategies. The incidence of cholangiocarcinoma, particularly iCCA, has increased globally over the past few decades. Surgical resection is the primary curative treatment for all subtypes, while liver transplantation after neoadjuvant chemoradiation is limited to early-stage pCCA. For advanced or unresectable disease, locoregional and systemic chemotherapeutics are the main treatment options. Advances in external-beam radiation therapy have improved treatment outcomes. Molecular profiling has identified promising targets for precision medicine, including immunotherapy. Biomarker-driven trials, stratifying patients by subtype and genetic aberrations, are essential for developing targeted therapies. Targeting the rich tumor stroma in conjunction with targeted therapies may also be beneficial. The article reviews the evolving developments in the epidemiology, pathogenesis, and management of cholangiocarcinoma.