Posterior cortical atrophy belongs to the progressive focal atrophy group of neurodegenerative diseases. It is characterized by specific clinical deficits in visual perception, which can affect either visuo-spatial features or identity of objects. The posterior cortical atrophy, initially described by Pick in 1902, is a rare and not well known syndrome. While it is easy to differentiate posterior cortical atrophy from Alzheimer's disease, some cognitive deficits are common with those of Lewy body disease and corticobasal degeneration, which results in diagnostic mistakes. This paper proposes a synthetic review of the posterior cortical atrophy illustrated by a clinical case.