Objective To investigate the clinical features of patients with cholestatic autoimmune hepatitis (AIH) and their prognosis after corticosteroid therapy. Methods The patients with AIH, autoimmune hepatitis-liver cirrhosis (AIH-LC), or autoimmune hepatitis-liver failure (AIH-LF) who were hospitalized in 302 Hospital of PLA from January 2014 to December 2015 were enrolled, and a retrospective analysis was performed for the clinical data of the patients with cholestatic AIH who met the inclusion criteria. The improvement rate of patients receiving corticosteroid therapy and reasons why corticosteroid therapy was not applied were analyzed. A one-way analysis of variance was used for comparison of normally distributed continuous data between multiple groups, and the Kruskal-Wallis H test was used for non-normally distributed continuous data between multiple groups; the chi-square test was used for comparison of categorical data between groups. Results A total of 181 patients with cholestatic AIH were enrolled, with 15 patients in AIH group, 142 in AIH-LC group, and 24 in AIH-LF group. The male/female ratio was 1∶11.1. Type-1 AIH patients with positive anti-nuclear antibody and/or anti-smooth muscle antibody accounted for 96.7% (175/181). Most patients with cholestatic AIH had liver cirrhosis (78.5%). There were significant differences between the three groups in age, albumin, prothrombin time activity, alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transpeptidase and total bilirubin(F=3.836, 13.271, and 41.819, χ2=29.153, 22.759, 8.826, and 26.942, all P＜0.05). The 181 patients with cholestatic AIH achieved an overall improvement rate of 36.5%, and the AIH group had the highest improvement rate (66.7%). Of all patients, 35 (19.3%) received corticosteroid therapy, and these patients had a significantly higher improvement rate than those who did not receive corticosteroid therapy (57.1% vs 31.5%, χ2=8.009, P=0.005). Infection, ascites, and gastrointestinal bleeding were main reasons for the absence of corticosteroid therapy. Conclusion Cholestatic AIH is commonly seen in female patients, and most of patients with cholestatic AIH have liver cirrhosis or type-1 AIH. Corticosteroid therapy is an effective method, but its application is limited by infection, ascites, and gastrointestinal bleeding. [ABSTRACT FROM AUTHOR]