Superficial siderosis of the central nervous system results from hemosiderin deposition in the subpial layers of the brain and spinal cord. A clinical history of subarachnoid hemorrhage is often absent. Patients present with slowly progressive gait ataxia and sensorineural hearing impairment. A history of prior intradural surgery or trauma is common. With widespread use of magnetic resonance imaging, presymptomatic cases are being diagnosed and it is difficult to be certain about the true incidence of this disorder. Despite extensive investigations, the cause of bleeding is often not apparent. An intraspinal fluid-filled collection is a common accompaniment and may be the likely bleeding source. An early diagnosis and prompt intervention directed at removal of the bleeding source may prevent progression. This review discusses the role of multimodality imaging in evaluation of superficial siderosis and the therapeutic implications of identified associations.