Epithelioid hemangioendothelioma (EH) is a rare borderline malignant tumor which originating from vascular endothelial cells and occurs in many organs such as soft tissues, lung, liver and bone. But, pulmonary EH which simultaneously involves pleura and liver is very rare. In the present report, we describe an uncommon case of EH involving the lung, pleura and liver in a 26-year-old woman. Chest CT showed single nodule in right upper lobe with large amount of pleural effusion at ipsilateral side and abdominal CT multiple round low densities in liver. The EH was confirmed by microscopic analysis and immunohistochemical staining of CD34+ from open lung biopsy specimen.