Synovial sarcoma is a type of malignant tumor with bidirectional differentiation of mesenchymal and epithelial cells,which can occur in any location.It mainly affects the deep soft tissues of the limbs,while pri-mary synovial sarcoma of the heart is very rare.The main clinical manifestations are corresponding symptoms caused by cardiac obstruction,embolism,and tamponade.When the site of occurrence is atypical,diagnosis is of-ten challenging,relying on histopathological morphology,immunohistochemistry,and molecular genetic examina-tion(SYT-SSX fusion gene changes)for diagnosis.We analyzed the clinical data,pathological features,treat-ment,and follow-up of a case of primary cardiac synovial sarcoma,and reviewed relevant literature to enhance the understanding of the disease among clinical and pathological physicians.