A 55-year-old right-handed woman presented initially with mild amnestic and depressive episodes but slowly developed progressive neurobehavioral symptoms, indicative of posterior cortical atrophy in ensuing years. A more detailed neurobehavioral test suggested predominant right temporo-parietal dysfunction with executive functional deficits. SPECT and MRI findings revealed right unilateral temporo-parietal involvement. Cholinesterase inhibitor administration led to amelioration of symptoms. We suggest that cases of posterior cortical atrophy or visual variant of Alzheimer's disease may be responsive to cholinesterase inhibitor therapy; however, the observation in a single case should be confirmed on larger populations in a clinical trial design with placebo control.