Epithelial hemangioendothelioma (EH), originally termed intravascular sclerosing bronchioloalveolar tumor (IVBAT), is a rare pulmonary vascular neoplasm usually associated with an indolent but slowly progressive clinical course. We describe a patient with EH who presented with alveolar hemorrhage and died within 8 wk. This report extends the clinical spectrum of epithelial hemangioendothelioma to include this aggressive form. EH should be considered in the differential diagnosis of alveolar hemorrhage syndrome.