Progressive visual complaints related to visuospatial disorders, and less often to visuoperceptual disorders, may be the presenting and isolated manifestation of a focal degeneration in the posterior cortical areas, called posterior cortical atrophy (PCA). PCA is a clinical syndrome corresponding to a focal variant of Alzheimer's disease in 80% of cases. The predominant dysfunction in the occipitoparietal pathways results in predominant visuospatial disorders, manifesting primarily as dorsal simultanagnosia, alone or associated with other symptoms of Balint's syndrome. PCA is rare and affects young patients who are fully aware of their deficits. Diagnosis of PCA is often delayed, due to insidious onset and development of symptoms, and to poor awareness of the condition in the medical community. An earlier diagnosis requires both better knowledge of PCA among ophthalmologists and neurologists and better recognition of visual complaints, leading to simple bedside tasks that can tackle the syndrome.