Objective To assess the effect of ursedeoxycholic acid (UDCA) withdrawal in primary biliary cirrhosis (PBC). Methods Twenty seven patients who were biochemically normalized for more than 6 months were derided to two groups. Group A discontinued UDCA, Group B received UDCA 13-15 mg'kg·-1·d-1.The two groups were well matched in age, sex, and disease duration. The patient# were followed-up for 12 months. Results Two patients withdrawal the therapy because of imcompliance. Therefore, there were 12 patients in Group A, 13 patients in Group B for analysis. Two patients (17%) showed disease progression in group A in 3 months and 12 months during follow-up respectively, which were indicated by the doubling of serum bilirubin concentrations above normal limits, and none in group B. Disease relapse rate was 92% and 15%, respectively, and the difference was significant (P<0.05). Alkaline phosphates (ALP), alanine aminotransferase (ALT), aspartate aminotransferase (AST), and γ-glutamy-hranspeptidase (GGT) deteriorated significantly, but serum bilirubin level remained unchanged. All other laboratory tests values remained unchanged. Conclusion long-term UDCA therapy is recommended in PBC patients, even though they achieve biochemical normalization.