Primary biliary cholangitis (PBC) is a complex disease resulting from the combination of genetic susceptibility, unknown environmental exposures and epigenetic alterations. The diagnosis is based on persistently elevated cholestatic liver function tests and PBC-specific serology. The majority of PBC patients are asymptomatic at diagnosis; when symptomatic, they usually report fatigue, pruritus, and dry eyes and mouth. The introduction of ursodeoxycholic acid (UDCA) has dramatically changed the disease course of individuals with PBC and reduced the rate of liver transplantation (LT). PBC has a relatively predictable natural history and accurate prognostic models have been developed. PBC is a good indication for LT considering the excellent long-term post-transplant survival, better than for other indications, although the disease recurs almost universally.