ObjectiveTo improve the knowledge of clinical features,image and pathology of Behcet's disease with the pulmonary artery aneurysm. MethodsOne typical female patient aged 40 years with recurrent pulmonary artery aneurysm caused by Behcet's disease was analyzed including clinical data,image and pathological features.The Pubmed (1967.1 to 2013.10) and Wanfang database (1982.1 to 2013.10) were searched with "Behcet's disease","pulmonary artery aneurysm" as search terms. ResultsRecurrent hemoptysis was major clinical manifestation of pulmonary artery aneurysm caused by Behcet's disease.Features of image including hilar enlargement,pulmonary artery aneurysm and primary thrombus.During the first hospitalization,the patient was diagnosed as pulmonary artery aneurysm.Then pulmonary lobectomy was performed and pathology examination revealed artery aneurysm and primary thrombus.During the second hospitalization,she was diagnosed as pulmonary thromboembolism and it was considered as failure of treatment of anticoagulant therapy.During the third hospitalization,she was diagnosed as pulmonary artery aneurysm of Behcet's disease,and recurrent hemoptysis was fully ameliorated by the combination treatment of glucocorticoid and immunodepressant. ConclusionPulmonary artery aneurysm is the typical feature of pulmonary vascular lesions of Behcet's disease,and recurrent hemoptysis is major clinical manifestation.CT angiography can identify location of pulmonary artery aneurysm.The key drugs of therapy are imunosuppressive drugs in combination with steroids.