A 69-year-old male patient presented with multiple lung nodules revealed by chest-computed tomography (CT) during a preoperative examination for an appendiceal tumor. The nodule diameters ranged from 2-10 mm without either pleural thickening or effusions. A fluorine-18-labeled fluorodeoxyglucose (F-FDG)-positron emission tomography (PET)/CT scan showed a high FDG uptake in the appendiceal tumor, but almost normal standardized uptake values in the bilateral lung nodules. A CT-guided biopsy led to a diagnosis of pulmonary epithelioid hemangioendothelioma, a rare vascular tumor with a radiological presentation similar to that of a metastatic lung tumor. The present case is the first to describe successful treatment using a CT-guided biopsy instead of more conventional methods.