A 45-year old woman with congestive heart failure due to left atrial tumor was operated on. Three tumor masses arising from posterior wall, atrial septum, anterior portion of mitral valve were resected. Pathological diagnosis was malignant fibrous histiocytoma [MFH]. Above one case and sixteen previous reports are reviewed. Eleven cases out of 17 were females. The tumors all originated primarily in the left atrium and 8 had distant metastases. The metastatic sites are lung [4 cases], brain [2 cases], liver, jejunum, cervix and pleura etc. Careful pathologic study is necessary to differentiate the uniformly fatal MFH of the heart from the more common benign atrial myxoma. The treatment modalities are surgical resection, chemotherapy, and radiation therapy & the prognosis of intracardiac MFH is poor. We underwent partial resection of left atrial MFH and obtained symptom relief and patient still alive 7 months post-operatively in state of NYHA class II.