Superficial siderosis is caused by recurrent haemorrhage in the subarachnoid space leading to haemosiderin deposition. It typically causes the triad of ataxia, deafness and myelopathy. We report a patient who developed superficial siderosis following neurosurgery for syringomyelia and who had an improvement in his hearing and mobility following treatment with a new iron chelation therapy that can penetrate the blood–brain barrier. It provides an intriguing insight into a therapy that could potentially modify the course of this rare neurodegenerative disorder. Further studies are required to assess the clinical efficacy of deferiprone in superficial siderosis.