We report on three patients with superficial siderosis of the central nervous system. The main clinical findings were progressive dementia, hearing loss and ataxia in combination with repeated xanthochromia of the cerebrospinal fluid. Diagnosis was made in one patient with magnetic resonance imaging (MRI), which showed a hyperintense rim around the surface of the cerebellum and the spinal cord. In the two other cases necropsy disclosed superficial iron and hemosiderin deposits on the surface of the brain. The etiology either was idiopathic or secondary to chronic intracranial bleeding by an angioma or after multiple head injuries. Superficial siderosis should be taken into account as one reason for dementia.