Pulmonary epitheloid hemangioendothelioma (PEHE) is a rare, often incidentally diagnosed, endothelial tumor of the lung. We present a case of a young adult who presented with acute hypoxemic respiratory failure and severe pulmonary hypertension with subsequent imaging and tissue biopsy confirming a diagnosis of PEHE. We briefly highlight the unique clinical, radiographic and histopathologic aspects of this rare disease. We propose that PEHE should be considered in the differential diagnosis for acute hypoxemic respiratory failure associated with bilateral pulmonary cavitary nodules and bronchoscopy should be considered as an initial diagnostic test.