Abstract: A case report where a Takayasu’s arteritis involving pulmonary artery was detected on pulmonary ventilation/perfusion imaging was presented. The characteristics of this case on the basis of clinical symptoms, signs, laboratory examinations and imaging examinations were analyzed. On the pulmonary ventilation/perfusion images, the "mismatches" were observed in the whole right lung, and the upper, posterior lobes, and basal segment of the left lung. Only from the results of pulmonary ventilation/perfusion examination, it is easy to be misdiagnosed as pulmonary embolism. While it is considered as a result of the pulmonary artery involvement in the Takayasu's arteritis, once the patient’s history and other examination results were analyzed, as well as the peer review of related literatures. In this case report, SPECT proved a non-invasive diagnostic tool for Takayasu's arteritis patient involved pulmonary artery. It offered precise diagnosis and thereby appropriate treatment strategies are taken on those patients.