Objective To enhance the recognition of pulmonary epithelioid hemangioendothelioma and heighten the ratio of diagnosis.Methods A case diagnosed with pulmonary epithelioid hemangioendothelioma by transbronchial biopsy was described and the related literature was reviewed.Results A 6l year old male patient was admitted to the hospital because of dry cough for over four months.CT scan showed multiple bilateral pulmonary nodules and the enlarged mediastinal lymph nodes.PET CT showed the nodule on left upper lobe and mediastinal lymph nodes with increased glucose metabolism.The final diagnosis was made by transbronchial biopsy pathology.Literatures showed that the etiology of PEH was not clear and most patients were asymptomatic.The characteristic feature of PEH on chest CT was the presence of multiple bilateral perivascular nodules,ground glass opacity,interlobular septal thickening,and calcification.The pathological characteristics typically included tumor cells arranging in nests and intracytoplasmic vacuoles.Some cells showed a signet ring-like shape.Immunohistochemistry showed that endothelium derived markers were positive,especially CD31,CD34.Conclusions Pulmonary epithelioid hemangioendothelioma is a rare tumor of the lung with non-specific clinical manifestations,difficult to diagnose,and easily misdiagnosed.