Cholangiocarcinomas (CCAs) are a group of hepatobiliary cancers with distinct anatomical subtypes: intrahepatic (iCCA), perihilar (pCCA), and distal (dCCA). These subtypes differ in their epidemiology, etiology, pathogenesis, and treatment. The incidence and mortality of iCCA have increased over the past three decades, with a low survival rate after diagnosis. Geographic variations in CCA incidence are related to differences in risk factors. Genetic and epigenetic alterations, as well as changes in oncogene and inflammatory signaling pathways, contribute to CCA development. The dense stroma surrounding CCAs, containing cancer-associated fibroblasts, promotes tumor progression. Advances in imaging and cytologic techniques have improved the detection and diagnosis of CCAs. Surgical resection is the primary treatment for iCCA, while liver transplantation after neoadjuvant chemoradiation is an option for pCCA. The article reviews recent advancements in the understanding of CCA epidemiology, pathogenesis, classification, diagnosis, and treatment.Cholangiocarcinomas (CCAs) are a group of hepatobiliary cancers with distinct anatomical subtypes: intrahepatic (iCCA), perihilar (pCCA), and distal (dCCA). These subtypes differ in their epidemiology, etiology, pathogenesis, and treatment. The incidence and mortality of iCCA have increased over the past three decades, with a low survival rate after diagnosis. Geographic variations in CCA incidence are related to differences in risk factors. Genetic and epigenetic alterations, as well as changes in oncogene and inflammatory signaling pathways, contribute to CCA development. The dense stroma surrounding CCAs, containing cancer-associated fibroblasts, promotes tumor progression. Advances in imaging and cytologic techniques have improved the detection and diagnosis of CCAs. Surgical resection is the primary treatment for iCCA, while liver transplantation after neoadjuvant chemoradiation is an option for pCCA. The article reviews recent advancements in the understanding of CCA epidemiology, pathogenesis, classification, diagnosis, and treatment.