Colangiocarcinoma (CCA) is a progressively fatal disease which generally occurs due to malignanttransformation of hepatic biliary cholangiocytes. The incidence of CCA has been increasing worldwide andthere is an urgent requirement for effective diagnosis and treatment strategies against this devastating disease.Different factors including liver-fluke infestation, viral hepatitis, exogenous nitrosamine-mediated DNA damage,and chronic inflammation have been linked to CCA genesis. However, the risk factors and underlying complexmechanisms leading to development of CCA are not sufficiently understood to devise an effective targetedtreatment therapy. In this review, we summarize currently known epidemiological and pathological aspectsof the disease and briefly describe various potential biomarkers and experimental anticancer phytochemicalsrelated to CCA research. In addition, we also sum up recent findings that link chronic inflammation of hepaticbiliary cholangiocytes with CCA. The collective information concisely presented in this article would provideuseful insights into the current understanding of this cancer.