The primary angiitis of the central nervous system (PACNS) is a rare vascular inflammatory disease that involving the brain and spinal cord. Currently, the diagnosis of PACNS is mainly dependent on clinical manifestations and signs, imaging, cerebrospinal fluid examination and brain biopsy. The magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) are employed in PACNS to assess the vascular lesions. Pathological diagnosis can be confirmed by brain biopsy. Retrospective studies have shown that cyclophosphamide combined with glucocorticoids can be used as a therapeutic regimen in the induction phase of PACNS, while immunosuppressive agents such as azathioprine can be used as maintaining therapy. In addition, the therapeutic benefits of biological agents such as rituximab, an anti-CD20 monoclonal antibody, have also been reported. However, due to the rarity and heterogeneity of PACNS, there is no definite clinical evidence related to diagnosis and treatment strategies, so the diagnosis and treatment of PACNS is still very challenging in clinical practice. Therefore, It is urgent to explore the pathogenesis of PACNS in order to provide scientific basis for early diagnosis and improvement of therapeutic effect.