BACKGROUND/AIMS: Autoimmune hepatitis (AIH) predominantly affects women. Reasons for this are unclear and few series have assessed long-term outcomes of men with AIH. METHODS: To evaluate the clinical course and outcomes of 51 men from a total of 238 consecutive patients with definite AIH at a single centre from 1971 to 2005. The primary outcome measure was death or liver transplantation. RESULTS: Median age at diagnosis was 39 y in men and 49 y in women (p = 0.0589). HLA A1, B8 and DR3 allotypes and the HLA A1-B8-DR3 haplotype were more frequently expressed in men (63% vs. 45%, p = 0.049; 74% vs. 38%, p < 0.001; 62% vs. 44%, p = 0.058; and 50% vs. 23%, p = 0.003; respectively). There were no significant differences in clinical manifestations at presentation. Over 96% of patients demonstrated a complete initial response to treatment. A greater number of men experienced at least one relapse (71% vs. 55%, p = 0.0591). However, women were significantly more likely to die or require liver transplantation (Log rank test p = 0.024). CONCLUSIONS: Men with AIH appear to have a higher relapse rate and younger age of disease onset which may relate to increased prevalence of HLA A1-B8-DR3. Despite this, men have significantly better long-term survival and outcomes than women.