A 56-year-old man was referred to our hospital for examination of a small pulmonary nodule revealed by chest X-ray. Contrast-enhanced chest computed tomography (CT) confirmed a solitary nodule of approximately 15mm in diameter in segment 8 of the left lung. ¹⁸F-fluorodeoxyglucose positron emission tomography (FDG-PET) showed no FDG uptake by the nodule. Transbronchial lung biopsy (TBLB) was performed to exclude the possibility of primary lung cancer. Pathological findings revealed a central hyalinized matrix and proliferation of epithelium-like cells. Immunohistochemical staining showed that tumor cells were positive for CD31 and CD34. Pulmonary epithelioid hemangioendothelioma (PEH) was diagnosed based on these findings. Clinical manifestation of PEH is variable; typically, patients are asymptomatic, and PEH is detected on routine chest radiographs as multiple small nodules in bilateral lungs. Diagnosis of PEH usually requires a surgical lung biopsy. Herein, we describe a rare case of PEH with a unilateral nodule that was diagnosed by TBLB.