Pulmonary artery sarcoma is a rare tumor that is frequently misdiagnosed as chronic pulmonary embolism. With heightened clinical awareness and advancement in technology, the diagnosis is now increasingly being made preoperatively. Previous literature has described the disease to be uniformly fatal, with surgical resection as the single most effective modality for short-term palliation. We present the case of a patient in whom pulmonary artery sarcoma was diagnosed preoperatively and who underwent surgical resection with no evidence of recurrence during long-term follow-up, suggesting that early identification and aggressive surgical intervention has the potential to be curative.