A 75-year-old man presented with dyspnea for more than 2 months, with blood test showing low platelet count and cardiac ultrasound showing severe pulmonary hypertension (>54 mm Hg). A CT pulmonary angiogram showed a filling defect in the pulmonary trunk, right and left pulmonary arteries, raising the possibilities of pulmonary embolism or artery sarcoma. FDG PET/CT was performed for further evaluation and showed low uptake in the pulmonary wall, which supported the diagnosis of pulmonary embolism. Patient was treated with anticoagulants with no changes on repeated CT pulmonary angiogram. Patient underwent surgery, and histopatological examination revealed a pulmonary artery sarcoma.