Cholangiocarcinoma (CCA) is a diverse group of biliary epithelial tumors, classified into intrahepatic (iCCA), perihilar (pCCA), and distal (dCCA) subtypes. Each subtype has distinct epidemiology, biology, and treatment strategies. The incidence of CCA, particularly iCCA, has increased globally over the past few decades. Surgical resection is the mainstay of potentially 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 chemotherapy are primary options. Advances in genetic profiling and targeted therapies, along with radiation and immunotherapy, are improving survival outcomes. The epidemiology of CCA varies geographically, influenced by risk factors and genetic differences. iCCA and pCCA/dCCA account for most CCA cases, with high incidence in northeast Thailand due to liver fluke infection. Incidence trends show increases in iCCA and decreases in pCCA/dCCA in some regions. Misclassification of CCA subtypes, such as pCCA being misclassified as iCCA, may skew reported rates. The incidence of CCA is also influenced by demographic factors like obesity and chronic viral hepatitis. Diagnosis of CCA involves imaging, biomarkers like CA 19-9, and molecular techniques. Surgical resection remains the primary treatment for early-stage CCA, while liver transplantation is considered for early-stage pCCA. For advanced disease, locoregional therapies like TACE and radioembolization are used. Systemic chemotherapy with gemcitabine and cisplatin is standard but has limited efficacy. Newer therapies, including targeted agents like FGFR inhibitors, PARP inhibitors, and EZH2 inhibitors, are being evaluated in clinical trials. Radiation therapy has evolved with advanced techniques like SBRT and proton therapy, improving treatment outcomes. Molecular profiling has identified genetic drivers in CCA subtypes, leading to targeted therapies. Epigenetic therapies, such as IDH inhibitors and HDAC inhibitors, are also being explored. These advancements highlight the need for personalized treatment strategies based on molecular subtypes and genetic aberrations. Ongoing research aims to improve survival and outcomes for patients with this aggressive malignancy.Cholangiocarcinoma (CCA) is a diverse group of biliary epithelial tumors, classified into intrahepatic (iCCA), perihilar (pCCA), and distal (dCCA) subtypes. Each subtype has distinct epidemiology, biology, and treatment strategies. The incidence of CCA, particularly iCCA, has increased globally over the past few decades. Surgical resection is the mainstay of potentially 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 chemotherapy are primary options. Advances in genetic profiling and targeted therapies, along with radiation and immunotherapy, are improving survival outcomes. The epidemiology of CCA varies geographically, influenced by risk factors and genetic differences. iCCA and pCCA/dCCA account for most CCA cases, with high incidence in northeast Thailand due to liver fluke infection. Incidence trends show increases in iCCA and decreases in pCCA/dCCA in some regions. Misclassification of CCA subtypes, such as pCCA being misclassified as iCCA, may skew reported rates. The incidence of CCA is also influenced by demographic factors like obesity and chronic viral hepatitis. Diagnosis of CCA involves imaging, biomarkers like CA 19-9, and molecular techniques. Surgical resection remains the primary treatment for early-stage CCA, while liver transplantation is considered for early-stage pCCA. For advanced disease, locoregional therapies like TACE and radioembolization are used. Systemic chemotherapy with gemcitabine and cisplatin is standard but has limited efficacy. Newer therapies, including targeted agents like FGFR inhibitors, PARP inhibitors, and EZH2 inhibitors, are being evaluated in clinical trials. Radiation therapy has evolved with advanced techniques like SBRT and proton therapy, improving treatment outcomes. Molecular profiling has identified genetic drivers in CCA subtypes, leading to targeted therapies. Epigenetic therapies, such as IDH inhibitors and HDAC inhibitors, are also being explored. These advancements highlight the need for personalized treatment strategies based on molecular subtypes and genetic aberrations. Ongoing research aims to improve survival and outcomes for patients with this aggressive malignancy.