A 69-year-old woman with a history of aortic dissection was referred to our department due to an abnormal chest X-ray. Contrast-enhanced computed tomography (CT) revealed a 60-mm heterogeneously enhanced mass in the left hilum and lesions seen as filling defects in the lumen of the main trunk and left lower branch of the pulmonary artery. Bronchoscopy revealed thrombus occlusion in the left upper lobe bronchus, and a large amount of Aspergillus sp. hyphae were detected in the lavage fluid, so we administered antifungal agents in combination with antibacterial agents. Intrapulmonary artery aspiration cytology of the lesion in the pulmonary artery and a thoracoscopic needle biopsy of the hilar lesion were performed, with a suspicion of pulmonary artery sarcoma. However, a definitive diagnosis could not be obtained in life. After treatment, the left hilar lesion reduced in size, whereas the intravascular lesions increased. The patient died of hemoptysis 6 months after her first visit. Pathological findings on autopsy revealed a pulmonary artery sarcoma localized in the pulmonary artery and granulomatous tissue in the left hilar lesion. Her clinical course and pathological findings strongly suggested a complication of pulmonary aspergillosis.