ObjectiveTo enhance the understanding of pulmonary epithelioid hemangioendothelioma (PEH), and improve the diagnostic rate of the disease. MethodsThe experience of diagnosis and treatment of a case of PEH in August 2013 was reported in the present study and the related literature was reviewed. The etiology, clinical manifestations, pathological diagnosis, differential diagnosis, therapy and prognosis of this disease were described. ResultsThe etiology of PEH remained unknown. Symptoms of PEH were usually nonspecific and mild. Chest radiograph or computed tomography usually revealed calcification in multiple nodules of half or both lungs. Primitive lumen formed by a single cell was the pathologic feature. Immunohistochemical stains showed that the malignant cells were of endothelial type. Genetic analysis was relatively limited currently. There was no effective treatment for this disease and the prognosis was poor. ConclusionPEH is a low potential malignancy occurring exclusively in young women. Symptoms of PEH are usually nonspecific and variable which can easily lead to misdiagnosis. So, pathological diagnosis should be emphasized.