Superficial siderosis (SS) of the central nervous system is a rare disorder in which the most common clinical manifestations are gait ataxia, sensorineural deafness and affection of the corticospinal tracts. Knowledge of this disorder is important since early diagnosis and treatment is crucial for the prognosis. Furthermore, new treatment strategies are emerging. The effect of corticosteroids on SS without an identifiable bleeding source has been debated. We present a case with a good clinical effect of steroids.