We describe 5 patients with complex visual disturbances in the absence of ocular pathology who were ultimately diagnosed with posterior cortical atrophy (PCA). The presence of visual cortical symptoms, neuroimaging findings and clinical evolution led to the diagnosis 1-5 years after the onset of visual symptoms. Age of onset ranged from 50-66 years. In 3 cases, magnetic resonance imaging (MRI) of the brain demonstrated predominantly right posterior cortical atrophy. The other 2 patients had nonspecific MRI findings but the diagnosis was established given the findings on clinical examination and positron emission tomography (PET). All progressed to global dementia and an autopsy confirmed the diagnosis of Alzheimer disease in one patient. The possibility of PCA should be considered when a patient presents with complex visual symptoms in the absence of ocular pathology. Early neurological assessment may avoid diagnostic delay.