Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is a very rare entity which is considered as a treatable condition. In some cases, clinical and radiological findings, associated to favorable evolution on steroids therapy can be sufficiently distinguishable to diagnose chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. We report the case of a 50-year-old man, suffering from acute dizziness with right facial paralysis and limited ocular abduction with his magnetic resonance imaging showing large confluent T2 and fluid-attenuated inversion recovery brainstem hyperintensities extending into the upper cervical spinal cord, infiltrating the basal ganglia and the thalami, with some punctate hyperintensities "peppering" the medial aspects of cerebellar hemispheres. This case illustrates atypical imaging features of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids on magnetic resonance imaging and our work also reviews different studies in the literature and highlights the differential diagnosis.