Objective To discuss the diagnosis of moyamoya disease with the 256 slices volumes perfusion CT angiography. Methods Selected 27 cases of Moyamoya disease patients as study,from the Third Affiliated Hospital of Qiqihar Medical University between January 2013 to December 2014, all patients had DSA diagnostic data, used Maximal Intensity Projection(MIP), volume rendering (VR), multiplanar reconstruction(MRP) and other methods to reconstruct image, while observing cerebral perfusion flow (CBF), cerebral blood volume (CBV), time to peek(TTP) to evaluate the supply with Moyamoya disease patients. Results 27 patients CTA image can show abnormal vascular stenosis, occlusion of the image, and DSA compare the diagnostic sensitivity and accuracy of 100%, while perfusion imaging found in blood supply and blood supply to reduce the vascular network area with the contralateral comparison reference area The results were statistically significant (P <0.05). Conclusion 256-slice CT image post-processing techniques intracranial vascular network and vascular abnormalities stenosis, occlusion, etc. can be a good method; brain perfusion imaging can clearly show the brain perfusion status, effective evaluation of dynamic changes in blood flow this method has a good prospect for moyamoya disease at diagnosis.