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Unlabelled: Autoimmune hepatitis/primary sclerosing cholangitis (AIH/PSC) overlap syndrome is a relatively uncommon variant of PSC., Aim: To evaluate the natural history of AIH/PSC overlap syndrome compared to a group of "classical" PSC., Methods: Forty-one consecutive PSC patients, with a regular follow-up of at least 2 years, were prospectively included in the study. Among these, 7 fulfilled the criteria for AIH/PSC overlap syndrome., Results: The AIH/PSC overlap group significantly differed from the "classical" PSC group in the following parameters: mean age at presentation (21.4 +/- 5.0 vs 32.3 +/- 10 years, p < 0.01), AST 191.0 +/- 14.8 vs 48.9 +/- 34.5 U/L, p < 0.005), ALT (357.0 +/- 26.5 vs 83.7 +/- 60.7 U/L, p < 0.005) and serum IgG (25.6 +/- 4.7 vs 12.9 +/- 6.0 mg/dl, p < 0.0001). The mean follow-up was similar in the 2 groups (93.3 +/- 65.9 vs 98.1 +/- 65.9 months respectively). Treatment included immunosuppression + ursodeoxycholic acid (UDCA) in the AIH/PSC overlap patients, and UDCA in the "classical" PSC group. Deaths were recorded only in the classical PSC group. The median survival in the latter group was 207 months (95% C.I. 87.6-326.4). The major events during the follow-up included: OLTx (1/7 vs 6/34), and neoplasms (only in the group of "classical" PSC). The new Mayo score prognostic index only increased significantly during follow-up in the "classical" PSC group (r2 0.8117, p < 0.01), Conclusion: Patients with AIH/PSC overlap syndrome seem to benefit from immunosuppression + UDCA therapy, survival is apparently better than in "classical" PSC condition.