Association of autoimmune disease in patients with primary biliary cirrhosis (PBC) was described. Forty-one patients with asymptomatic PBC (a-PBC) and 13 patients with symptomatic PBC (s-PBC) were examined. The overall prevalence of autoimmune disease in association with PBC was 63%. There was no significant difference in the frequency of autoimmune disease association between a-PBC (63.4%) and s-PBC (61.5%). The most frequent complication was Sjögren's syndrome (31.7% in a-PBC and 38.5% in s-PBC). Raynaud's phenomenon was seen in 7 patients (12.2% in a-PBC and 15.4% in s-PBC). Five of these 7 patients showed the CREST syndrome having the anti-centromere antibody. Association of 3 polyarthritis, 2 autoimmune hepatitis, 2 Hashimoto's disease, 1 systemic lupus erythematosus, 1 polymyositis and 1 temporal arteritis was seen in a-PBC patients.