The CLIPPERS syndrome is a rare inflammatory disease of the central nervous system, which is based on lymphocytic inflammation of the pons Varolii. Diagnosis of this pathology is a difficult task and relies primarily on neuroimaging and immunological methods. The key diagnostic criteria also include regression of the pathological process after treatment with glucocorticosteroids. In this literature review, special attention is paid to the features of therapy and differential diagnosis of the CLIPPERS syndrome with two clinically similar diseases of the central nervous system: primary vasculitis of the central nervous system and the PRES syndrome.