An unusual presentation of pulmonary epitheloid hemangioendothelioma.

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.