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Pulmonary artery sarcomas (PAS) are rare and probably incurable tumours. The clinical manifestations are non-specific and very similar to that of patients with thromboembolic disease, resulting in delay of the correct diagnosis and proper treatment. We report the case of a 66-year-old woman with PAS diagnosed by computed tomography guided biopsy. Chemotherapy treatment was initiated but the patient died 11 months after diagnosis.