Abstract: Objective To observe the clinical feature and curative efficacy of AIH-PBC overlap syndrome.Methods Study 1: Clinical features of 124 patients with primary biliary cirrhosis, 57 patients with autoimmune hepatitis and 39 patients with AIH-PBC overlap syndrome were retrospectively analyzed.Study 2: Curative efficacy of 39 patients with AIH-PBC overlap syndrome were grouping analyzed based on different treatment schedule.Results AIH-PBC overlap syndrome constituted 17.73% of 220 patients with autoimmune liver diseases.No significant differences were found in sex ratio and symptoms of fatigue and jaundice among three groups.But there were significance in age and symptom of pruritus among them (P<0.05) .The levels of ALT, AST, ALP and GGT had significant differences.ANA, AMA, Gp210 and SP100 were found in AIH-PBC overlap group and the positive rates of AMA, Gp210 and SP100 were remarkably higher than AIH group whereas the rate of SMA was higher than PBC group (P<0.05) .The pathological findings in the overlap syndrome showed combined features of AIH and PBC.Whatever UDCA added or not added in glucocorticoids, level of ALP and GGT decreased after therapy.In combined group, the improvement of ALT, AST, GLB and IgG got significance (P<0.05) .Biochemical responses in terms of AIH features (ALT decline to normal, IgG≤16 g/L) were achieved in especially combined group.Although more alleviation of inflammatory infiltration and damage of bile ducts appeared in combined group, adverse reaction such as fracture or alimentary tract hemorrhage were also higher observed in this group.Conclusion AIH-PBC overlap syndrome showed combined features of both PBC and AIH and there were unique characteristic of its own.Combination of UDCA and glucocorticoids is benefit for achieving better biochemical response and pathological improvement for AIH-PBC overlap syndrome.