Cholangiocarcinoma (CC) is the second most common primary hepatic malignancy, accounting for 10-25% of all hepatobiliary malignancies. The incidence varies significantly by geographic region, with higher rates in Asia compared to Western countries. Established risk factors for CC include parasitic infections, primary sclerosing cholangitis, biliary-duct cysts, hepatolithiasis, and toxins. Other potential risk factors include inflammatory bowel disease, hepatitis C virus, hepatitis B virus, cirrhosis, diabetes, obesity, alcohol consumption, smoking, and genetic polymorphisms. The distinction between intrahepatic cholangiocarcinoma (ICC) and extrahepatic cholangiocarcinoma (ECC) is important, as they may have different epidemiological features and risk factors. Misclassification of Klatskin tumors and the lack of a consistent classification system have contributed to the complexity of understanding CC risk factors. Studies suggest that diabetes and heavy alcohol consumption may increase the risk of CC, while the role of IBD, HCV, HBV, and cirrhosis varies by region. The consistent use of refined classification would improve the understanding of CC risk factors.Cholangiocarcinoma (CC) is the second most common primary hepatic malignancy, accounting for 10-25% of all hepatobiliary malignancies. The incidence varies significantly by geographic region, with higher rates in Asia compared to Western countries. Established risk factors for CC include parasitic infections, primary sclerosing cholangitis, biliary-duct cysts, hepatolithiasis, and toxins. Other potential risk factors include inflammatory bowel disease, hepatitis C virus, hepatitis B virus, cirrhosis, diabetes, obesity, alcohol consumption, smoking, and genetic polymorphisms. The distinction between intrahepatic cholangiocarcinoma (ICC) and extrahepatic cholangiocarcinoma (ECC) is important, as they may have different epidemiological features and risk factors. Misclassification of Klatskin tumors and the lack of a consistent classification system have contributed to the complexity of understanding CC risk factors. Studies suggest that diabetes and heavy alcohol consumption may increase the risk of CC, while the role of IBD, HCV, HBV, and cirrhosis varies by region. The consistent use of refined classification would improve the understanding of CC risk factors.