Anti-transcriptional intermediary factor-1?(TIF-1?) autoantibody may be associated with cancer in adult patients with dermatomyositis. The aim of this study was to evaluate the risk of cancer in the presence of anti-TIF-1? autoantibody in adult dermatomyositis. A comprehensive database search of EMBASE, MEDLINE and the Cochrane Library up to May 2018 was performed using the main key words? dermatomyositis?,?? myositis?,? inflammatory myopathies? and? anti-TIF-1?. Eighteen studies, with a total of 1,962 dermatomyositis, were included in the meta-analysis. The pooled prevalence of cancer-associated dermatomyositis in patients with anti-TIF-1? autoantibody was 0.41 (95% confidence interval (CI) 0.36? 0.45). In the presence of anti-TIF-1? autoantibody, the overall diagnostic odds ratio of cancer was 9.37 (95% CI 5.37? 16.34) with low heterogeneity (Cochran? s Q: 14.88 (df?=? 17, p?=? 0.604); I2?=? 0%). The results of this systematic review confirm that detection of anti-TIF-1? autoantibody is a valuable tool to identify a subset of adult dermatomyositis patients with higher risk of cancer.