Primary intimal sarcoma of the pulmonary artery is a rare disease and there has been no report of any case originating from the pulmonary valve. Recently we experienced a 62 year-old female patient who had a primary intimal sarcoma of the pulmonary valve with distal metastasis. She was brought to medical attention due to exertional dyspnea facial edema productive coughing and general weakness for 1 month. Chest CT and echocardi-ography suggest an acute pulmonary thromboembolism or tumor. Exploration showed a large polypoid mass arising from the pulmonary leaflets and multiple masses on distal pulmonary arteries. We replaced the pulmonary valve and reconstructed the pulmonary artery. She received radiotherapy 1 month postoperatively and now 4 months after surgery she has begun receiving chemotherapy.