Chronyc lymphosytic inflammation with pontine perivascular enhacement responsive to steroids (CLIPPERS), described for the first time in 2010 by Pittock and collaborators, is a rare inflammatory disease of the central nervous system (SNC) characterized by the presence of punctuate or curvilinear lesions described like “salt and pepper” appearance on the magnetic resonance imaging (MRI), that enhance with Gadoliniumn administration; this lesions show an exquisite response to corticosteroid therapy. The etiology of this disease remains unknown. However, the existing articles suggest an autoimmune component, which may or may not be related to other autoinmmune, infectious or malignant pathologies. Due to the above, it is generally considered that in order to reach the diagnosis of CLIPPERS, the possibility of other more common or aggressive pathologies must first be ruled out through extensive investigation. However, if the clinical and imaging presentation are typical and there is a very high suspicion of CLIPPERS, early initiation of glucosteroid therapy may be recommended. There is no unanimous therapy plan due to the few cases reported worldwide thus far. In cases of severe relapses or atypical behavior, both clinical and radiological, it is recommended to consider a stereotactic biopsy of the lesion to integrate the definitive diagnosis. Because it is a rare disease, it is relatively unknown to first- contact medical personnel; in our particular case, this leads to referral to the neurosurgery department who, thanks to their experience, have considered a demilinizing disease as a diagnostic possibility and requested an assessment by the neurology department. Here, we report the evolution and management of three diagnosed cases of CLIPPERS at a single Third-level Medical Center in México; based on clinical, radiological and neuropathological findings; as well as highlighting the importance of lesion biopsy in selected cases.