Malignant fibrous histiocytoma (MFH) of the heart is a very rare disease. It is difficult to distinguish from myxoma which is most common benign cardiac tumor. Prognosis of malignant fibrous histiocytoma (MFH) of the heart is known to be fatal despite of medical and surgical treatment in contrast to benign tumors. A primary myxoid malignant fibrous histiocytoma of the left atrium was diagnosed in a 58 years old woman. The mass was removed incompletely by operation. The patient did not receive either chemotherapy or radiotherapy. However, the patient is still alive for more than fifteen months after the initial presentation. We report a case of malignant fi brous histiocytoma (MFH) with reviewing the current references.