Autoimmune cholangitis is an entity with a controversial nosological location. It has been defined as a setting of autoimmune chronic cholestasis, clinically, biochemically and histologically resembling primary biliary cirrhosis, although with a different immunological profile. It lacks antimitochondrial antibodies and instead usually presents other autoimmunity features, basically positivity to antinuclear antibodies. In this article, we discuss its nosological location based on descriptions published on this entity and comparisons with primary biliary cirrhosis.