Background: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory disorder of the central nervous system characterized by distinctive punctate and curvilinear pontine gadolinium-enhancing lesions on magnetic resonance imaging (MRI) and a marked response to corticosteroid therapy. Owing to its rarity and overlap with several neurological disorders, diagnosis is often challenging. Case Report: A 45-year-old man presented with recurrent brief episodes of right-sided hemisensory paresthesia involving the face, upper limb, and lower limb without altered consciousness or post-event neurological deficits. Neurological examination was unremarkable. MRI brain revealed characteristic punctate enhancing lesions involving the pons and midbrain with additional supratentorial white matter involvement. Cerebrospinal fluid analysis and autoimmune, vasculitic, and demyelinating workup, including aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies were negative. Based on clinico-radiological findings and a dramatic response to corticosteroids, a diagnosis of CLIPPERS was established. Conclusion: CLIPPERS should be considered in patients with atypical recurrent brainstem-related symptoms and characteristic MRI findings after exclusion of close mimics. Early recognition is essential, as prompt corticosteroid therapy can result in favourable outcomes.