Superficial siderosis is a rare condition characterized by hemosiderin deposition in leptomeninges, subpial tissue, brainsten, cerebellum, spinal cord, and cranial nerves. Slowly progressive hearing loss and gait ataxia are invariable clinical manifestations. We report one patients with clinical and radiological features. Patinet presented with hearing loss and cognitive dysfunction. Patient showed typical MRI finding: marked linear hypointensities around the cerebellum, brainstem, and the surface of the cerebral cortex. Patient had no definite bleeding source. Hemosiderin deposition is caused by chronic and recurrent subarachnoid hemorrhage derived from tumor, vascular malformation, aneurys, posthemispherectomy, and unknown bleeding sources. Diagnosis is easily made by characteristic clinical manifestations and MRI findings. The selevtive vulnerability of the cerebellum and the 8th cranial nerve depends upon their own histological and biochemical characteristics. Benefits of the iron chelating agents are questionable. Removal of the possible bleeding sources is the most reliable strategy to prevent the disease progression.