A 30-year-old woman (daughter) was diagnosed to have primary biliary cholangitis (PBC) with autoimmune hepatitis (AIH) overlap syndrome. Although she was started on prednisolone and ursodeoxycholic acid (UA), she eventually died of hepatic failure with gastrointestinal hemorrhage seven months after the initial hospitalization. A 60-year-old woman (mother) was diagnosed with PBC with alcoholic liver cirrhosis, was treated with UA, and had no disease progression. These familial PBC patients had different clinical courses. While the mother was negative for the anti-glycoprotein 210 (anti-gp210) antibody, the daughter was positive for the same. These findings suggest that anti-gp210 antibody positivity affects the prognosis of PBC, even in familial cases.