Introduction: We present two cases with symptoms of progressively worsening cough, dyspnea, decreased exercise tolerance and right-sided back pain in the first case and upper respiratory symptoms characterized by cough and a low grade fever in the second case. Methods: Report of two cases. Results:The initial chest X-ray in both the cases showed pleural effusion. Further imaging with computed tomography of the chest confirmed the effusion in both cases. Thoracentesis was done in both of them revealed an exudative effusion that did not reveal any infection or malignancy. Both cases underwent surgical biopsy and the diagnosis of primary pleural epithelioid hemangioendothelioma was made. Conclusions: Both the cases had progressive clinical deterioration despite chemotherapy with Taxol and Bevacizumab in one case and carboplatin, etoposide, and bevacizumab, in the second case. Both developed metastatic disease to lungs and died.Please cite this paper as: Lazarus A, Fuhrer G, Malekiani C, McKay S and Thurber J. Primary pleural epithelioid hemangioendothelioma (EHE) -two cases and review of the literature. Clin Respir J 2011; 5: e1-e5.