We report the case of a woman with persistent bilateral pleural effusion and multiple bone metastases. Primary pleural epithelioid angiosarcoma was diagnosed on the basis of endothelial markers expressed on atypical cells from the pleural effusion. Despite intensive chemotherapy, anti-angiogenesis treatment, and surgical pleurodesis, the disease progressed and the patient died. The immunohistochemical stain of endothelial markers is valuable in confirming a diagnosis of angiosarcoma. The prognosis of primary pleural epithelioid angiosarcoma is particularly poor. It is hoped that there will be more effective treatment for this malignancy in the near feature.