Objectives. To estimate the number of patients with polymyositis/dermatomyositis (PM/DM) in Japan and the prevalence rate and incidence rate of the disease.Methods. The electronic database in the nationwide registration system on intractable diseases from 2003 to 2010 was utilized to identify the number of prevalent and incident cases of PM/DM. The electronic data entry rate was used to establish the total number of registered cases.Results. The estimated total number of patients with PM/DM and the prevalence rate in Japan in 2010 were 17,000 and 13.2 per 100,000 population, respectively. The prevalence of PM/DM ranged from 10 to 13 per 100,000 population with a trend toward increasing over time. The incidence of PM/DM was estimated within the range 10–13 per 1,000,000 person-years, except for 2003.Conclusions. We report the prevalence and incidence of PM/DM recently in Japan for the first time at the nationwide population level. Because the prevalence seems to be increasing recently, continued monitoring of these epidemiologic features is required.