Superficial siderosis (SS), as a result of chronic subarachnoid haemorrhage and haemosiderin deposition on the leptomeninges and subpial layers of the brain, cerebellum and spinal cord, can cause ataxia, pyramidal tract lesions and hearing deficits. In cases with not pronounced hearing impairment adult-onset spinocerebellar ataxia can be considered as a differential diagnostic alternative. We report a similar case where the diagnosis of SS was established by means of gradient echo MRI sequences 5 years after symptom onset. A bleeding lumbar ependymoma was identified as a source of haemorrhage. Surgical tumor resection stopped any further disease progression. Our report underlines that clinicians should be aware of the clinical features and diagnostic pitfalls of SS. Time of diagnosis and neurosurgical intervention can essentially influence the patients' prognosis.