Cholangitis is a serious life-threatening situation affecting the hepatobiliary system. This review provides an update regarding the clinical and pathological features of various forms of cholangitis. A comprehensive search was performed in the PubMed, Scopus, and Web of Knowledge databases. It was found that the etiology and pathogenesis of cholangitis are heterogeneous. Cholangitis can be categorized as primary sclerosing (PSC), secondary (acute) cholangitis, and a recently characterized form, known as IgG4-associated cholangitis (IAC). Roles of genetic and acquired factors have been noted in development of various forms of cholangitis. PSC commonly follows a chronic and progressive course that may terminate in hepatobiliary neoplasms. In particular, PSC commonly has been associated with inflammatory bowel disease. Bacterial infections are known as the most common cause for AC. On the other hand, IAC has been commonly encountered along with pancreatitis. Imaging evaluation of the hepatobiliary system has emerged as a crucial tool in the management of cholangitis. Endoscopic retrograde cholangiography, magnetic resonance cholangiopancreatography and endoscopic ultrasonography comprise three of the modalities that are frequently exploited as both diagnostic and therapeutic tools. Biliary drainage procedures using these methods is necessary for controlling the progression of cholangitis. Promising results have been reported for the role of antibiotic treatment in management of AC and PSC; however, immunosuppressive drugs have also rendered clinical responses in IAC. With respect to the high rate of complications, surgical interventions in patients with cholangitis are generally restricted to those patients in whom other therapeutic approaches have failed.