A 40 year-old female was examined for complaints of left arm pain and restriction of movement in the left upper extremity for 3 months. Chest X-ray showed a mass in the left upper lung and the patient was evaluated with computed tomography that confirmed the significant mass in the left paratracheal region and also showed small nodules in both of the lungs. A whole-body FDG-PET scan was performed for the suspicion of malignancy. FDG-PET indicated high FDG accumulation in the lung lesions mainly in the left paratracheal region. FDG-PET findings were highly suspicious of malignancy so the patient had thoracoscopic biopsy of the lesion. The histological findings and immunohistochemistry tests were consistent with pulmonary epitheloid hemangioendothelioma (PEH). Epitheloid hemangioendothelioma (EH) is a systemic name that represents a rare type of malignant tumor of vascular endothelial origin, which can arise in bone, liver, soft-tissue, or lung. PEH is currently known as the lung form of EH. Consequently, our patient had resection of the left paratracheal mass. This report presents a rare case of histologically confirmed PEH, which showed increased FDG accumulation on FDG-PET study. PEH should be added to the other causes of increased FDG uptake.