BACKGROUND There is no satisfactory medical treatment for patients with primary sclerosing cholangitis. There are conflicting data regarding the clinical benefit of high doses of ursodeoxycholic acid (UDCA) in primary sclerosing cholangitis. OBJECTIVE To evaluate using meta-analysis, if UDCA (standard or high-dose) is useful in primary sclerosing cholangitis. METHODS We searched MEDLINE using the textwords 'PSC', 'treatment', 'UDCA' and retrieved all abstracts from the major Gastroenterology and Liver meetings. We included randomised clinical trials comparing standard or high-dose of UDCA (>15 mg/kg body weight per day) vs. placebo or no intervention. End-points: mortality or liver transplantation, pruritus, fatigue, cholangiocarcinoma and histological progression. RESULTS We identified eight randomised clinical trials comprising 567 patients. Five used standard doses and three high doses of UDCA. There was no significant difference in mortality [OR, 0.6 (95% CI, 0.4-1.4)], in pruritus [OR, 1.5 (95% CI, 0.3-7.2)], in fatigue [OR, 0.0 (95% CI, 0.1-7.7)], in cholangiocarcinoma [OR, 1.7 (95% CI, 0.6-5.1)] and in histology stage progression [OR, 0.9 (95% CI, 0.34-2.44)]. No differences were found in the subgroup analyses. CONCLUSIONS Neither standard nor high-dose UDCA influence favourably the progression of primary sclerosing cholangitis.