To investigate the pathogenesis, etiology, clinical manifestation, imaging characteristics, and treatment of superficial siderosis of the central nervous system (SSCNS). Methods: One Case of SSCNS diagnosed by imaging, biopsy pathology, and clinical methods was included. Clinical data of 62 patients in China were extracted from the China National Knowledge Internet, Wanfang, and China Science and Technology Journal Database. The cases were reviewed, classified, and analyzed by descriptive statistical analysis. Results: The major clinical manifestations of the case were sensorineural hearing loss in both ears, cerebellar ataxia, cognitive impairment, and pyramidal sign. SSCNS was finally diagnosed as classical infratentorial superficial siderosis of unknown etiology. Magnetic resonance imaging (MRI) showed characteristic marginal hypo-intensity around the central nervous system, and pathological biopsy showed Prussian blue positive staining on the pia mater. All 62 patients mainly presented with a combination of sensorineural hearing loss (52/62), cerebellar ataxia (55/62), pyramidal sign (40/62), and other typical SSCNS triads, with corresponding imaging changes. However, the majority (43/62) were of unknown etiologies. Nine patients received iron chelation therapy, while six received surgical intervention, with varying outcomes. Conclusion: SSCNS is a rare degenerative disease mainly diagnosed by a combination of iron-sensitive MRI sequences and typical clinical manifestations. Although brain biopsy can further support the diagnosis, there remains no effective treatment to date.