Primary angiitis of the central nervous system (PACNS) is an uncommon immune-mediated inflammatory disease in which lesions are limited to the small and medium blood vessels of the brain parenchyma, spinal cord and pia mater. Because of its various clinical and neuroimaging manifestations, PACNS has been considered in the differential diagnosis of many diseases. The diagnosis of PACNS is challenging, and the complementation of angiography and brain biopsy is helpful in the diagnosis. Compared with the foreign counterparts, few cases of PACNS had been reported in China, and the level of diagnosis and treatment are far from satisfaction. Therefore, it is urgent to promote the understanding of PACNS among Chinese medical care providers. This paper focuses on the content of Chinese expert consensus on the diagnosis and treatment of PACNS, and combines with the clinical diagnosis and treatment experiences in PACNS, in order to improve the understanding of PACNS.