A 66-year-old male presented with rhabdomyosarcoma of the right kidney with inferior vena cava tumor thrombus and with tumor emboli to right pulmonary artery and two large fragments of tumor thrombus free floating in the right atrium. The patient underwent right radical nephrectomy and thrombectomy of the right atrium and pulmonary artery. He was discharged on postoperative day 13. To our knowledge this is the first case of large tumor embolism to the right atrium and pulmonary artery from renal rhabdomyosarcoma reported in the literature.