Synovial sarcoma comprises approximately 10% of all soft tissue sarcoma diagnoses; a primary synovial sarcoma of the myocardium is exceedingly rare. There have been very few cases reported in the literature thus far. With the identification of the characteristic and diagnostic chromosomal abnormality t(X;18), this may become an increasingly recognized entity. Our report adds to the limited published cases of primary cardiac synovial sarcoma with the characteristic t(X;18). Further elucidation of the effects of this translocation on the cell cycle may lead to directed therapies in the future.