To investigate the existing evidence on the effectiveness of approaches to treating inclusion body myositis and to assess the methodological quality of this evidence. The Cochrane Controlled Trials Register (CENTRAL), Medline, Embase, Cinahl, Physiotherapy Evidence (Pedro), McMaster and Web of Science databases were searched. The references of identified articles and reviews were also checked for relevancy. The methodological quality was assessed according to the Cochrane Collaboration's domain-based evaluation framework. Of the 331 identified records, 10 were considered relevant for a qualitative analysis. The risk of bias was considered being low for six studies and high for four. Eight studies were randomized controlled trials, and two were controlled clinical trials. In the samples, male gender predominated, and the mean age of the participants varied from 51 to 72 years. The duration of intervention varied from 3 to 17 months. One small trial on the effect of oxandrolone reported a significant positive result. The other trials observed no improvement or insignificant improvement among the participants treated with intravenous immunoglobulin, methotrexate, etanercept or interferon. Thus far, there is no evidence indicating that any specific treatment is the effective in treating inclusion body myositis.