Objectives Pericardial effusion is a rare clinical manifestation in idiopathic inflammatory myopathies (IIMs). It has been described in a small number of literature studies worldwide. We describe the clinical and laboratory characteristics of 19 IIM patients combined with pericardial effusion, and compare them with previously reported cases. The single-center observational-study-inspired collected of 156 IIM patients with complete data from January 1, 2016 to January 1, 2021 in the First Affiliated Hospital of Xinjiang Medical University, of which 19 patients had pericardial effusion. Methods The clinical characteristics of 19 IIM patients complicated with pericardial effusion were investigated by descriptive analysis and compared with previously reported cases. Results 19 cases of IIM patients had pericardial effusion (12.2%), patients without a large amount of pericardial effusion or pericardial tamponade. There was a predominance of women in the patients with 78.9% pericardial effusion . In the clinical examination, 10 cases showed chest tightness (52.6%), pulmonary fibrosis (47.4%), and the frequency of muscle nuclear magnetic, which suggested that muscle lymphocyte infiltration rate was 63.2%. Anti-Ro-52 antibody and anti-Jo-1 antibody were positive (26.3%, 42.1%). IIM patients with pericardial effusion were accompanied by decreased serum albumin levels and elevated ESR. In the literature review, the most common clinical characteristics of IIM patients with pericardial effusion were female, pulmonary fibrosis, shortness of breath, positive anti-Ro-52 pulmonary fibrosis, and anti-Jo-1 antibody. Conclusion In the study, 19 patients of IIMs with pericardial effusion present with chest tightness, and are accompanied by pulmonary fibrosis, positive anti-Jo-1 antibody, and anti-Ro-52 antibody. It is suggested that pericardial effusion in IIM patients may be related to anti-synthetase antibody.