Pulmonary epithelioid hemangioendothelioma (PEHE) is a rare vascular tumor of endothelial origin first described in 1975 as intravascular bronchioloalveolar tumor. Since then, >100 cases have been reported, and most cases require surgical lung biopsy for diagnosis. We report the case of a 46-year-old man with a diagnosis of PEHE from endobronchial biopsies of an intraluminal nodule, a rare presentation of this disease. We summarize a review of the literature and the bronchoscopic findings of PEHE.