To clarify the clinical features and CT findings of IgG4-related sclerosing cholangitis (IgG4-SC), we reviewed 16 cases of IgG4-SC and 10 cases of cholangiocarcinoma concerning patient background, treatment, outcomes and CT findings. The median age of IgG4-SC cases was 70 (range 54-79) years, and only 1 was a woman. Serum IgG4 level of all IgG4-SC patients were elevated and in 13 patients steroid therapy proved effective. The CT findings were analyzed with regard to the biliary strictures (symmetry, outer margin, inner margin), wall enhancement pattern and pancreas size. The CT findings of symmetric wall thickness and total scores were significantly higher in IgG4-SC than in cholangiocarcinoma. Although the small number of patients in this study is a limitation, the CT findings may help distinguish IgG4-SC from cholangiocarcinoma.