This case describes the evaluation and surgical excision of a rare intraluminal primary pulmonary myxoid sarcoma of the pulmonary artery. A 28-year-old woman presenting with dyspnea, hemoptysis, and weight loss was found to have a mass occluding the right pulmonary artery. Determined by imaging and biopsy to be an exceedingly rare, low-grade tumor, the patient underwent surgical resection with curative intent. The tumor and was totally resected and the pulmonary arteries reconstructed with bovine pericardial homograft. Post-operatively the patient reported resolution of symptoms and proceeded to routine monitoring.