We report a 53-year-old man who presented with dyspnea and low-grade fever. Cardiac ultrasound showed pulmonary hypertension and an ill-defined echogenic mass within the pulmonary trunk. Computed tomography scan revealed an inhomogeneous mass which filled the main pulmonary trunk with near-total occlusion, and extended into both pulmonary arteries. Anticoagulant therapy was administered based on a presumptive diagnosis of pulmonary thromboembolism. Positron-emission tomography scan was useful for differentiating the mass, which was determined as a pulmonary artery sarcoma by surgical resection. Although complete resection was impossible, the patient survived for 20 months with adjuvant chemotherapy and medical treatment.