CASE PRESENTATION: A 73 year old male presented with right chest pain, and his chest CT scan showed an irregularly mass in the superior lobe of right lung and multiple nodular thickening of the right pleura. Percutaneous lung biopsy guided by CT showed epithelioid hemangioendothelioma. Immunohistochemical stains was positive for CD31, F8, and vimentin and was negative for Calretinin. Following tissue diagnosis, the chemotherapy regimen of pemetrexed combined with vascular endostatin Endostar was administered, but the regiment didn’t benefit the patient. His condition continued to deteriorate and only survived for four months following diagnosis.