Clinical significance of anti-Saccharomyces cerevisiae antibody (ASCA) and its prevalence in Chinese primary biliary cirrhosis (PBC) patients have not been characterized and therefore needs to be defined. Enzyme-linked immunosorbent assay was used to test ASCA in sera from 198 PBC patients, 85 patients with other liver diseases (OLD) and 35 health controls (HC). Indirect immunofluorescence was used to detect anti-mitochondrial antibodies (AMA) in PBC. Results showed that the frequency of ASCA in PBC, 29.8 %, was higher than other disease groups. And ASCA occurred more frequently in PBC patients with positive anti-gp210 than the negative ones. Also, ASCA was detected in 7 out of 15 PBC negative for AMA. Some liver-related biochemical indices and inflammatory indices were significantly higher in PBC patients with positive ASCA (p < 0.05). In conclusion, the prevalence of ASCA in Chinese PBC patients is 29.8 %. PBC patients with positive ASCA are associated with more severe liver injury, and ASCA-IgA might be related to disease activity of PBC.