The objective of this chapter is to introduce the biliary pathology. Biliary Disease affects the bile ducts, gallbladder and other parts involved with bile. Etiologies of biliary diseases are primary biliary cirrhosis and primary sclerosing cholangitis. Chronic cholestatic autoimmune disorder characterized by nonsuppurative granulomatous cholangitis effecting septal and interlobar bile ducts. Primary sclerosing cholangitis (PSC) is a chronic, progressive, cholestatic liver disease resulting from inflammation, fibrosis, and destruction of the intrahepatic and extrahepatic bile ducts. This leads to multiple areas of stricturing in the biliary tree and eventually to cirrhosis.