Because of its rarity and the similarity of its presentation to that of pulmonary thromboembolic disease, the diagnosis of pulmonary artery sarcoma is often not considered early in patients presenting with recurrent or chronic pulmonary emboli. We present a case of pulmonary artery sarcoma that was treated as pulmonary embolism for 3 years before surgical resection was carried out. Two years after the resection the patient is well with no clinical or radiologic evidence of recurrent or metastatic disease.