Objective　To discuss the clinical and imaging features of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Methods　Clinical characteristics, imaging features, treatment, prognosis, and follow up data from one pediatric patient diagnosed CLIPPERS were analyzed retrospectively. Results　 Female, 12 years old, presented with limb weakness, gait ataxia, and right visual field defect. Neurological signs mainly presented with oculomotor palsies, nystagmus, the left facial nerve palsy and cerebellar ataxia. Brain magnetic resonance imaging (MRI) showed appearance with punctate and curvilinear gadolinium enhancement 'peppering' the pons, medulla oblongata, bilateral arm, and the left side of the midbrain. After treatment with steroids, the clinical and imaging manifestations were rapidly improved. Conclusions　Pediatric patient of CLIPPERS may have more atypical presentations. The diagnosis of CLIPPERS mainly depends on imaging and treatment response.