Primary biliary cholangitis and autoimmune hepatitis are common autoimmune diseases of the liver. Both have typical clinical presentations, including certain autoantibodies on serologic testing. Histologic features are also often typical: primary biliary cholangitis shows bile duct destruction (sometimes with granulomas), and autoimmune hepatitis shows prominent portal and lobular lymphoplasmacytic inflammation. Both have a wide differential diagnosis, including one another; they may also simultaneously occur within the same patient. Careful use of clinical and histologic criteria may be necessary for diagnosis. First-line therapy is immunosuppression for autoimmune hepatitis and ursodeoxycholic acid for primary biliary cholangitis. Both diseases may progress to cirrhosis.