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Giant cell arteritis (GCA) combined with concomitant pulmonary embolism (PE) is extremely difficult to diagnose because of its low incidence and atypical clinical presentations.A 62-year-old male developed fever of unknown origin.Positron emission tomography/computed tomography (PET/CT) revealed increased glucose metabolism in the vascular walls of the ascending and descending aorta and pulmonary artery, leading to a diagnosis of GCA combined with PE.The patient did not respond to regular antiviral and antibacterial treatment but was remised after steroid treatment.No specific autoantibodies were positive for this patient, and the patient did not respond to regular antiviral and antibacterial treatment. After diagnosed by PET/CT, the patient responded well to steroid treatment. Literature review found 16 cases of GCA diagnosed by PET/CT. Their median age was 68.5 (range, 21–87) years and 13 cases were female. PET/CT showed significantly increased metabolism in the ascending and descending aorta, abdominal aorta, and carotid artery. In 4 cases (including our own case), the mean maximum standardized uptake value was 4.2 ± 1.7 (range, 2.5–7.2). Six cases of GCA also had PE and 5 (6/7, 85.7%) cases were females, and the current case is the first male case of GCA combined with PE. Steroid therapy was initiated in all 5 cases. Complete remission was achieved in 4 cases and 2 patients died and the outcome of 1 patient was unknown.Our case and the review highlight the value of PET/CT in diagnosing GCA combined with PE, suggesting that PET/CT is the preferred diagnostic tool for atypical patients presenting with fever or muscle pain.