The aim of this study was to analyze the computed tomography (CT), fluorine-18 fluorodeoxyglucose positron emission tomography/ computed tomography ( 18 F-FDG PET/CT), and clinical findings of pulmonary epithelioid hemangioendothelioma.Methods: Eighteen patients were included. We reviewed CT and PET/CT features and clinical information. Spearman correlation analysis was used to reveal the correlation between lesion size and maximum standardized uptake values (SUVmax).Results: Eleven patients complained of different symptoms. The serum levels of tumor markers were elevated in 8 patients (44.4%). Multiple lesions were shown in 14 cases (77.8%). Eleven cases (61.1%) presented as bilateral multiple perivascular lesions. The size of the largest nodule was less than 20 mm in diameter in 11 cases (61.1%). Eleven cases underwent PET/CT examination, and 8 cases (72.7%) had increased FDG uptake. There was a positive correlation (Spearman correlation coefficient, 0.56; P = 0.009) between lesion size and SUVmax. Extrapulmonary involvement was shown in 7 cases, including the liver and bone.Conclusions: Pulmonary epithelioid hemangioendothelioma on CT scan usually showed multiple bilateral perivascular lung nodules, generally measuring less than 20 mm in diameter. Lesions usually showed increased FDG uptake on PET/CT scans. There was a positive correlation between lesion size and SUVmax. The liver and bone were the most frequently involved extrapulmonary organs.