Primary malignant cardiac tumors occur extremely rarely. Among these, leiomyosarcomas are exceptionally rare. We described a case of left atrial leiomyosarcoma in which surgical intervention was followed by adjuvant radiation therapy. A 74-year-old male was admitted for dyspnea. Chest X-ray showed severe pulmonary congestion. Echocardiography revealed large tumor in the left atrium. Emergency operation was performed. The tumor invaded the left atrial wall and the mitral valve, and the lesion was resected as extensively as possible. Postoperative pathologic examination confirmed leiomyosarcoma. He underwent adjuvant radiotherapy postoperatively. However, early local recurrence was recognized. He died due to sudden circulatory collapse in 8th postoperative month. As cardiac leiomyosarcomas have extremely poor prognosis, complete resection and effective postoperative adjuvant therapy are necessary.