The prevalence of autoimmune liver disease (AiLD)-related autoantibodies has not been defined in Chinese patients with primary biliary cirrhosis (PBC) and therefore needs to be characterized. The purpose of this study was to explore the prevalence of AiLD-related autoantibodies in Chinese patients with PBC. Indirect immunofluorescence was used to detect anti-mitochondrial antibodies (AMA) in the sera from 198 PBC, 44 autoimmune hepatitis (AIH) and 41 non-autoimmune liver disease controls (LDC). ELISA assays were used to test the presence of anti-M2, anti-gp210, anti-sp100, anti-SLA, anti-LC1 and anti-LKM-1 antibodies. AMA was present in 92.4, 15.9 and 7.3%, respectively, of patients with PBC, AIH and LDC. Anti-M2 was present in 87.4% of the PBC patients, but only in 4.5 and 4.9%, respectively, of AIH and LDC patients. Anti-gp210 and anti-sp100 were detected in 34.3 and 25.8% of PBC, 6.8 and 15.9% of AIH and in 4.9 and 17.1% of LDC patients. Anti-LC1, anti-SLA and anti-LKM-1 were, respectively, present in 1.5, 0.0 and 0.5% of PBC, and 9.1, 11.4 and 2.3% of AIH, and none of LDC. PBC patients that were AMA-positive presented with higher IgM levels but lower CRP levels than negative ones. ALT, TBIL, DBIL, GGT, ALP, and AST were detected at higher levels and ALB at lower levels in PBC positive for anti-gp210 (P<0.05). The prevalence of AiLD-related autoantibodies we provided might help define PBC in China. Anti-sp100 is not a better prognostic marker for Chinese PBC patients compared to anti-gp210. The identification of effective diagnostic biomarkers for AMA-negative PBC patients is still needed.