A 71-year-old right-handed man (F.G.) presents with prosopagnosia and with an inability to recognize famous and familiar buildings. Despite his deficit, F.G. obtained normal scores on neuropsychological tests of executive functions, language, praxis and primary visuoperceptual skills. Brain MRI showed atrophy predominantly in the right temporal lobe, particularly in the fusiform gyrus and the parahippocampal cortex. The present study investigated F.G.'s ability to orient himself in familiar and new environments. His wayfinding abilities in a familiar environment (i.e., his hometown) were preserved despite an inability to recognize familiar and famous buildings, monuments and landmarks in this environment. Wayfinding was achieved through a heavy reliance on written indications (e.g., names of restaurants and streets), preservation of a pre-existing cognitive map of this familiar environment, and normal executive functions necessary to plan the execution of a given trajectory. In an unfamiliar environment, F.G.'s topographical agnosia resulted in severe wayfinding difficulties and in the inability to build an adequate cognitive spatial representation. F.G.'s topographical agnosia results from a high-level visuoperceptual deficit, characterized by an inability to access a global configuration of complex visual stimuli such as familiar and famous monuments, and an over-reliance on the processing of local features.