Objective To describe the clinical characteristics of polymyositis/dermatomyositis (PM/DM) with anti-aminoacyl-tRNA synthetase (ARS) antibody positive. Methods The clinical, laboratory and radiographic results of PM/DM patients hospitalized in our department from September 2014 to November 2017 were retrospectively analyzed. Results A total of 39 patients were diagnosed (14 cases positive for anti-Jo-1 antibody, 10 cases positive for non-anti-Jo-1 ARS antibodies, and 15 negative for ARS antibodies). The frequency of ARS antibodies positive patients who had interstitial lung disease was higher than those patients without ARS antibodies (P<0.05). Amyosthenia and mechanic's hand were more common in the patients with anti-Jo-1 positive (P<0.05) and the frequency of clinical amyopathic dermatomyositis in non-anti-Jo-1 positive patients was significantly higher (P<0.05). Conclusions The clinical characteristics are similar between anti-Jo-1-positive and non-Jo-1 ARS antibodies positive patients. Most PM/DM patients carrying anti-Jo-1 antibodies with interstitial lung disease own typical imaging characteristics of nonspecific interstitial pneumonia overlap organizing pneumonia (NSIP/OP). It can be diagnosed of non-anti-Jo-1 antibody syndrome although there is no clinical manifestation of myositis and anti-jo-1 antibody is negative.