Primary myxofibrosarcoma of the heart is a rare malignancy. To the best of our knowledge, no more than 17 cases have been reported since 1963.We report the case of a 42-year-old man who presented with dyspnea and palpitation of one month's duration. Echocardiography and enhanced computed tomography revealed a large tumor in the left atrium.The patient underwent palliative excision of the tumor and histopathological analysis revealed it to be a low-grade myxofibrosarcoma. The patient developed a bone metastasis two years later and is still alive 26 months after the cardiac surgery.Primary cardiac myxofibrosarcoma involves predominantly the left atrium and the most common symptom induced by the tumor is dyspnea. Disease outcome is often disappointing despite aggressive postoperative radiotherapy and chemotherapy.