This report describes an extremely rare cause of a common presentation. A 59-year-old man who presented with symptoms of progressive cardiac failure, was demonstrated to have a large left intraatrial mass on computed tomography pulmonary angiography and an echocardiogram. He proceeded to urgent surgery under cardiopulmonary bypass. Histology of the resected mass diagnosed primary pleomorphic sarcoma of the left atrium. This exceedingly rare malignancy has a poor prognosis and often presents with nonspecific symptoms. Echocardiography remains vital in diagnosis, and treatment is primarily surgical excision, with some reports of adjuvant chemotherapy.
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