Background: Patients with superficial siderosis (SS) rarely show brachial multisegmental amyotrophy with ventral intraspinal fluid collection accompanied with dural tear.
Case Presentation: We describe spinal cord pathology of a 58-year-old man who developed brachial multisegmental amyotrophy with ventral intraspinal fluid collection from the cervical to lumbar spinal levels accompanied with SS, dural tear, and snake-eyes appearance on magnetic resonance imaging (MRI). Radiological and pathological analyses detected diffuse and prominent superficial deposition of hemosiderin in the central nervous system.