We describe two primary biliary cholangitis (PBC) cases with hepatocellular carcinoma (HCC) in a non-cirrhotic stage. Case 1 : A 61-year-old woman was diagnosed as having PBC by anti-mitochondrial antibody (AMA) positivity. She was complicated with HCC at the age of 80, as confirmed by imaging studies along with elevation of tumor markers. Transcatheter arterial chemoembolization (TACE) therapy did not prevent a recurrence and she died at 82 years of age. A liver specimen at autopsy revealed Nakanuma stage 3 PBC with HCC dissemination. Case 2 : A 61-year-old woman was diagnosed at Nakanuma stage 3. She was complicated with HCC for 10 years in a non-cirrhotic stage but cured by TACE therapy. Clinicians should bear in mind that patients with an advanced but non-cirrhotic stage of PBC may become complicated with HCC. Further studies are needed to clarify the risk factors of HCC development in non-cirrhotic PBC.