Posterior cortical atrophy (PCA) is a neurodegenerative condition characterized by a progressive loss of visual processing skills and other posterior functions. Diagnosis is often delayed in PCA as symptoms can be difficult for the patient to articulate and for the clinician to detect. Diagnosis is particularly challenging in the earliest stages of the disease since visual symptoms are often mistaken as being related to ocular rather than cortical dysfunction. This report describes a 61-year-old man who volunteered as a healthy control participant in a longitudinal research study and was followed up for 5 years. During that time he showed a gradual decline in posterior cortical functions including visuoperceptual, visuospatial, and literacy impairments in the context of intact verbal episodic memory. Structural image analysis revealed atrophy which was initially most marked in inferior temporal and posterior parietal cortices before spreading to occipital cortices and subsequently to more anterior regions. Based on the clinical, neuropsychological and neuroimaging features, a diagnosis of PCA was made. The present case represents a unique opportunity to study and visualize the evolution of PCA from the very earliest symptomatic stages.