A case of myxoid liposarcoma of the left atrium in a 35-year old man is reported. The tumour, revealed by an atrial flutter, was excised. The patient died 13 months later, with multiple metastases. A review of the literature yielded only 7 cases of primary liposarcoma of the heart, including 4 which were surgically treated. This confirms the extreme rarity of a tumour which has a very poor prognosis due to recurrence in situ or metastatic spread.