Pulmonary embolism and splenic infarction are rare in patients with polycythemia vera. We herein describe a man in his early 60s whose main symptoms were chest tightness, cough, and sputum expectoration. Antibiotics, bronchodilators, and mucoactive agents did not improve his symptoms. Pulmonary artery computed tomography angiography showed pulmonary embolism, and abdominal computed tomography showed multiple hypodense foci in the spleen. Bone marrow aspiration cytology, biopsy, and genetic testing confirmed polycythemia vera. The patient’s symptoms were relieved after treatment with hydroxyurea and rivaroxaban. This case emphasizes that although pulmonary embolism and splenic infarction are relatively rare in patients with polycythemia vera, the possibility of polycythemia vera should be considered in clinical practice.

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