Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a newly described inflammatory disease of the central nervous system. The diagnosis is based on the clinical, radiological and neuropathological findings. Prominent response to steroids is also a characteristic diagnostic feature of the disorder. We present a case of a patient with probable CLIPPERS disease as well as discuss treatment and differential diagnosis. The patient received chronic immunosuppressive treatment with the use of steroids and steroid-sparing agent (methotrexate), which led to clinical and radiological improvement. There are no treatment guidelines due to the limited number of such cases. The knowledge of this disorder is important for neurologists and radiologists.