The hepatobiliary system is essential for digestion and includes: the liver, pancreas, bile ducts and the gallbladder. Many disorders and diseases can affect these organs of hepatobiliary system. Immunology deals with the defence mechanisms including all physical, chemical and biological properties of the organism that help it to combat its susceptibility to foreign organisms, material, etc. Many types of liver-resident antigen-presenting cells have immune regulatory function and they can also induce the differentiation of circulating immune cells into regulatory cells. Immunological diseases of the hepatobiliary system and the pancreas include a broad spectrum of disorders that manifest characteristic histopathology/serology and variable clinical features and imaging findings. Recent studies have thrown fresh light on the complex role of genetics and autoimmunity in the pathogenesis and natural history of these diverse disorders that include autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis, IgG4-related cholangitis, overlap/outlier syndromes, and autoimmune pancreatitis.