Dermatomyositis is a connective tissue disease mainly involving striated muscles and skins. It is rare to complicate with massive pericardial effusions. In this article, we tracked a 2-year treatment history of a dermatomyositis patient with rare complications. In the late stage of the disease, massive pericardial effusion was combined. Complications such as dysphagia, pulmonary hypertension, Sjogren's syndrome and anticardiolipin antibody syndrome posed great challenges to treatment. Massive pericardial effusion and pulmonary failure resulted in death of the patient, and anticardiolipin antibody syndrome was also one of the possible important causes of death. Good compliance could help control the disease. Massive pericardial effusion should be punctured in time. When combined with dysphagia, adequate intravenous immune globulin (IVIG) was the key to treatment.