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To explore the etiology, clinical manifestation, diagnosis, differential diagnosis, management and prognosis of pleural epithelioid hemangioendothelioma.The clinical data of a patient with pleural epithelioid hemangioend othelioma admitted to Peking Union Medical College Hospital were retrospectively analyzed and the related literature was reviewed. We searched databases PubMed, Embase, Ovid, Cochrane, Wanfang and Chinese National Knowledge infrastructure (CNKI) using the keyword "pleural epithelioid hemangioendothelioma" by December 2013.The patient was a 40-year-old male presented with chest pain, and his chest CT scan revealed thickening of the left pleura and left pleural effusion. Biopsy of the pleura showed epithelioid tumor cells, and immunohistochemistry was positive for CD31, CD34 and vimentin. From January 1975 to December 2013, 18 related articles were retrieved and 29 cases of pleural epithelioid hemangioendothelioma were reported. Among all 30 cases, there were 20 males and 10 females, ranging from 31 to 82 years old, and the average age was 50. 3 years old. The etiology of the disease remained unknown. Chest pain, cough, and dyspnea were the common symptoms. Computed tomography usually revealed pleural effusion and pleural thickening or mass. Histological examinations revealed mainly epithelioid cells. Immunohistochemical stains were positive for vascular endothelial markers. The mean survival time was 8.2 months.Pleural epithelioid hemangioendothelioma is rare and the etiology is unknown. Clinical and imaging manifestations are not specific, and diagnosis is relied on histological findings. It should be differentiated from adenocarcinoma, hemangiosarcoma, mesothelioma and pulmonary epithelioid hemangioendothelioma. There is no effective treatment and its prognosis is poorer than its pulmonary counterpart.