A primary malignant fibrous histiocytoma of the left atrium was diagnosed in a 27 year old woman. After surgical excision the tumour recurred together with enlargement of the right hilar lymph nodes. The patient was then treated with nine courses of chemotherapy using a combined drug regimen. During the first course the tumour regressed, and after nine courses almost complete remission was achieved. Subsequently, the residual tumour was removed by resection of the right lung, the right hilar, paratracheal, and paraeosophageal lymph nodes and by cardiotomy with partial resection of the right and left atria and atrial septum followed by a reconstruction of the atrias. To date, more than two years after initial presentation, the patient is alive and well.