Primary cardiac leiomyosarcoma is a rare tumor with poor prognosis. We present a 53-year-old female patient with biatrial leiomyosarcoma with inferior vena cava and coronary sinus involvement who underwent aggressive surgical resection with reconstruction of her left and right atrium and coronary sinus with bovine pericardium followed by adjuvant radiation therapy. She is living well with no recurrence 9 years after diagnosis.
