Herein, we describe a case of 69 year old man who presented with gradual bilateral hearing impairment and unsteadiness. Examination showed bilateral sensorineural deafness and cerebellar ataxia. Neuroimaging revealed a rim of hypointensity surrounding the brain stem, superficial cerebellar fissures and extending into the cervical spinal cord consistent with the diagnosis of superficial siderosis (SS). Investigations failed to identify any reversible cause for the condition.