Epithelioid hemangioendothelioma, originating from the vascular endothelium, is a very rare and low-grade malignancy. World-wide, about 50 cases of pulmonary epithelioid hemangioendothelioma have been reported. This is more common in female and is usually shown as multiple nodules in both lung fields. A 41-year-old male, who had suffered from right pleuritic chest pain for 3 months, was initially diagnosed as adenocarcinoma under bronchofiberscopic biopsy. At that time, the stage of tumor according to the TNM staging was llla. He received bronchoscopic biopsy again during follow-up period and it was diagnosed as hamartoma. After surgery, the final diagnosis was pulmonary epithelioid hemangioendothelioma.