We describe a patient who selectively lost the ability to orient himself in the environment after a stroke involving the right parahippocampal gyrus. The neuropsychological assessment showed a specific pattern of impairment of topographical memory; the patient recognised and recalled environmental landmarks but was unable to recall their spatial location. This study provides evidence that different forms of topographical disorientation may be related to distinct mechanisms of cognitive dysfunction. Furthermore, neuroimaging data suggest that a lesion of the right parahippocampal gyrus is critically related to pure topographical disorientation.