Primary tumors of the heart are relatively uncommon and their clinical sympfomatology is nonspecific mimicking many different cardiac diseases. Twenty-five percent of primary heart tumors are malignant. Among malignant primary heart tumors, 53 cases of rhabdomyosarcoma have been reported in literature. The development of echocardiographic technique has made it possible to screen large numbers of paftients with cardiac tumors. A primay cardiac rhabdomyosarcoma was successfully resected after the diagnosis was made by eehocardiography alone, Pathologic diagnosis was the pleomorphic type stage I. The patient was recommended to have combined multimodality treatment including postoperative chemoradiotheraphy. We presented the case and reviewed the literature.