The clinical presentation of Alzheimer's disease (AD) is heterogeneous, especially in individuals with an early age at onset, where the presenting symptoms may be language, behavior or visual impairment. Posterior cortical atrophy (PCA) refers to a syndrome where visual processing and other posterior functions are the initial symptoms. While the majority of PCA cases reflect the underlying AD, a proportion of cases are caused by dementia with Lewy bodies, corticobasal degeneration or prion disease. PCA is sometimes not recognized until late into the disease or misattributed to anxiety or malingering owing to its low frequency, the early age at onset and the relatively atypical symptoms. This article describes the PCA syndrome, emphasizing its clinical features and how it relates to early-onset AD variants. In order to aid identification of this syndrome, the neuroimaging and neuropsychological features, as well as biomarkers of the underlying pathology, are also described briefly. Finally, the implications for treatment, compensatory strategies and rehabilitation, as well as management of comorbidities, are discussed.The clinical presentation of Alzheimer's disease (AD) is heterogeneous, especially in individuals with an early age at onset.Presenting symptoms of AD may include primary impairments of language, behavior or vision.Posterior cortical atrophy (PCA) is characterized by progressive impairment in visuospatial and visuoperceptual function, alexia, and features of Balint's and Gerstmann's syndrome.The majority of PCA cases are due to AD histopathology; however, dementia with Lewy bodies, cortical basal degeneration and prion disease can all lead to a similar syndrome.In PCA, gray matter atrophy is seen on T 1 MRI in parietal, occipital and occipitotemporal cortices.Biomarkers such as cerebrospinal fluid, amyloid-b:tau ratio and amyloid PET imaging can indicate the underlying pathology.It is important to identify the underlying pathology in view of future protein-specific treatments and trials.It is important to differentiate clinical syndromes in order to provide carers and patients with relevant information and appropriate compensatory or rehabilitative strategies.