Primary biliary cirrhosis (PBC) is very closely associated with the presence of antimitochondrial antibodies (AMA). Nevertheless, 5 to 10% of patients with typical features of primary biliary cirrhosis do not have detectable AMA. They are referred to as AMA negative PBC. We report a case of PBC who was AMA-negative. The patient was a middle-aged woman seeking help of dermatologist for her complaint of itching. The diagnosis was confirmed by histopathology, whereas other possible causes of cirrhosis were excluded.