Abstract: Autoimmune liver diseases (AILD) represent a group of chronic, immune-mediated liver injuries with unknown etiology. The pathogenesis of these diseases remains elusive, and their clinical manifestations are non-specific, posing significant challenges for accurate diagnosis and long-term threats to human health. Therefore, the pathogenesis of AILD was reviewed, aiming to provide novel targets and robust evidence for clinical diagnosis and treatment of AILD.