Abstract Background context Superficial siderosis is a rare condition resulting from the presence of chronic bleeding into the subarachnoid space usually causing gait instability and deafness. The surgical management of superficial siderosis depends on localizing the source of hemorrhage. Purpose The surgical treatment of this rare condition has not been well described in the literature. We present a case illustrating the surgical treatment for superficial siderosis. Study design Case report. Patient sample The authors describe the case report of a 70-year-old gentleman with gait instability and deafness found to have an abnormal communication between the spinal epidural venous plexus and the subarachnoid space. Methods The source of hemorrhage into the subarachnoid space was identified to be a fistula in the ventral thoracic dural. A costotransversectomy approach was undertaken at the T4–T5 level to expose the fistula. The abnormal communication was patched and sealed. Results The patient's symptoms remained stable on follow-up at 15 months with no worsening of his symptoms. Conclusions Superficial siderosis is a neurologic disorder that arises from chronic hemosiderin deposition into the subarachnoid space. The progressive nature of the disease can be halted if a source of hemorrhage can be found and treated surgically.