Superficial siderosis of the central nervous system (SS) is a rare clinical syndrome due to repeated intracranial hemorrhage. We report a case of SS occurring 19 years after subtotal removal of a non-functioning pituitary adenoma. The patient was a 37-year-old female, exhibiting progressive bilateral sensory neural hearing loss and cerebellar ataxia. T2 weighted magnetic resonance imaging demonstrated a rim of low intensity signal on the cerebellum, brain stem, and sylvian fissure. Immediate diagnosis based on characteristic symptoms and MRI findings is important for the prevention of irreversible progression of SS.