We report the case of a 41-year-old male presenting with acute heart failure due to myxofibrosarcoma of the left atrium. Total resection was performed, followed by 4 courses of postoperative chemotherapy. Recurrence of the tumor was found 14 months later and resection was repeated with local irradiation and chemotherapy. Unfortunately, this failed to halt the spread of the malignancy and the patient died of brain metastasis. We conclude that cardiac myxofibrosarcoma is rare and highly malignant. Adjuvant chemotherapy and radiotherapy seemed ineffective in this case.
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