A patient developed a neurological disorder at the age of 57 years and died 9 years later after progressive aggravation. The clinical picture was that of a unilateral, parietal, focal neurological syndrome for many years, finally becoming bilateral. The purely neurological nature of the condition, without signs of mental disturbance, should be emphasized. Pathological examination showed mainly unilateral circumscribed cortical atrophy in the parietal regions. Histological examination showed atrophic lesions typical of Pick's disease. This case provides suggestive evidence for the existence of purely parietal forms of Pick's disease.