Scientific Background: Primary degenerative dementia can show different features in the early stages and can start with asymmetric or symmetric focal degeneration. Posterior cortical atrophy is a rare progressive dementia syndrome which can be evaluated, using neuropsychological tests and neuroimaging techniques, along with clinical features. Definitive diagnosis is made histopathologic ally. Objective: To discuss the clinical features of a case with posterior cortical atrophy, characterized by progressive visuospatial disorder, and the differential diagnosis, under the light of related literature. Results: Neurocognitive assessment revealed a serious visuospatial and constructional disturbance as well as facial recognition defects, dressing apraxia and agraphia, and a mild memory and verbal fluency impairment. Cranial MRI demonstrated bilateral parieto-occipital atrophy, and the SPECT investigation showed decreased perfusion in the right temporoparieto occipital region compared to the left hemisphere. Routine and other laboratory investigations were normal. Conclusion: The patient was started on memantine with a daily dose of 20 mg, twice a day. After four months, he became temporarily able to drive and park his tractor, but, the symptoms progressed later. (Archives of Neuropsychiatry 2009; 46: 192-6)