OBJECTIVES: To estimate the cost of treatment of primary biliary cirrhosis with ursodeoxycholic acid (UDCA) in Brazil and to evaluate the efficacy and safety of this treatment. METHODS: We considered the doses of 8, 12 and 15mg/Kg and a patient weighing 70Kg to estimate the cost of treatment. We used the maximum price to the producer in the CMED (Drug Market Regulation Chamber) list of 03/15/2013 with 18% of ICMS (Circulating Goods and Services Tax), and applied CAP (Adequacy Coefficient of Prices)of 25% . We used Purchasing Power Parity of 1,0USD=1,8BRL. To access efficacy and safety we searched the databases The Cochrane Library, CDR, Tripdatabase, MEDLINE and LILACS to identify systematic reviews (SR) of clinical trials that reported data on mortality, biochemical improvement measurements and adverse events. RESULTS: The estimated annual cost of treatment was USD2,239.24 with the dose of 8mg/Kg; USD3,168.97with the dose of 12mg/Kg; and UDS4,098.71 with the dose of 15mg/Kg. We included seven SRs; four evaluating UDCA versus placebo/observation; one UDCA versus colchicine; one UDCA versus methotrexate; and one UDCA versus bezafibrate. Generally, until four years of treatment there were no difference between UDCA and placebo/other interventions with respect with mortality, hepatic transplant incidence, worsening or arising of itching and fatigue, and incidence of hepatic complications. In the other hand there were improvements in surrogate outcomes like hepatic function markers, especially bilirubin. In all studies UDCA was well tolerated by patients. CONCLUSION: Improvements in the blood levels of hepatic markers did not match mortality rates or the incidence of transplant. The main symptoms of the disease, itching or fatigue, were not altered by the use of UDCA. There is a lack of evidence of studies evaluating quality of life of the patients, which perhaps could be improved by the use of UDCA. Besides, long observational studies could connect biochemical improvement and mortality rate.