Superficial siderosis of the central nervous system (SS) consists of intracellular and extracellular deposition of hemosiderin in the leptomeninges, subpial tissue, spinal cord and cranial nerves [1,2]. It is a result of chronic or recurrent extravasation of blood into the subarachnoid space, of capillary or venous origin, with a time course ranging from 3 to more than 20 year duration [3]. The majority of the neuropathological cases described in the literature are secondary to repeated haemorrhages from tumours, vascular malformations and subdural haematomas [4,5]; however, in about 40% of cases the source of bleeding remains unknown [6]. Patients with cavernous malformations adjacent to cerebrospinal fluid spaces are at increased risk for development of SS [7]. A triad of sensorineural hearing loss (95%), cerebellar ataxia (88%), and pyramidal signs (76%) characterizes SS [6]. Less frequent findings include dementia, headache, tremor, anosmia, bladder disturbance, nystagmus, and ocular motility deficits [8–10]. MRI of the brain represents a reliable method for the non-invasive diagnosis of SS. High-field T2-weighted spin-echo and gradient-echo MRI detect hemosiderin deposits by documenting the hypointense signal along the gyri, the ependymal surface of the ventricles, the surface of the brain stem and the spinal cord. This appearance is caused by magnetic susceptibility effects of the blood degradation products (ferritin and hemosiderin) [4,11,12]. We report a patient with typical neuroradiological features of SS associated with parkinsonism. The patient, an 81-year-old man, presented with parkinsonism associated with bilateral sensorineural deafness, ataxia and signs of corticospinal tract involvement. He had started, 2 years earlier, with progressive bilateral hearing loss, rigidity and bradykinesia on the left arm. Neurological examination revealed symmetrical cogwheel rigidity in all extremities, body-bradykinesia. The patient also exhibited a slightly unstable broad-based gait with some postural instability. The patient’s voice presented hypophonic with slurred speech and almost complete loss of prosody. The tendon reflexes were symmetrically hyperactive, with flexor plantar responses. He had no history of neuroleptic medication exposure or other noxious substances that could be implicated. Neuropsychological abnormalities in terms of memory, concentration and visuomotor tasks skills were not present. Treatment with carbidopa/levodopa with up to 100/1000 mg/day produced mild improvement of symptoms. The addition of tolcapone and pramipexole to levodopa/carbidopa treatment did not produce benefit. Magnetic resonance imaging (MRI) of the brain was performed at 2 T and revealed a cavernous malformation adjacent to the body of the right lateral ventricle measuring, approximately, 2 cm in diameter (Fig. 1). In addition, gradient-echo magnetic resonance and T2-weighted images demonstrated marginal hypointensity along the surface of the brainstem, cerebellar vermis and cerebellar folia, leading to the diagnosis of SS of the central nervous system (Fig. 2). The appearance of the substantia nigra was normal.