A detailed neuropsychological evaluation was performed on a patient with an idiopathic cerebellar degenerative disorder. Significant deficits were found in verbal and nonverbal intelligence, verbal associative learning, and visuospatial skills. These deficits were not readily explained by motor control difficulties. In contrast to the patient's moderately impaired language abilities, he was severely impaired on a test of verbal fluency and demonstrated mild naming deficits. Severe cerebellar parenchymal volume loss was demonstrated by magnetic resonance examination. Supratentorial structures showed only minimal posterior parietal and occipital sulcal prominence. On neurological examination, this patient had signs of severe involvement of the cerebellar systems and mild-to-moderate dysfunction of the corticospinal tract. As is characteristic of patients with cerebellar degeneration, there was neurophysiological evidence of subclinical involvement of auditory and somatosensory pathways at the level of the brain stem. Since relatively little cerebral cortical atrophy was noted in this patient, these findings suggest that an intact cerebellum is important for normal cognitive functions.