We describe a patient who, after right hemisphere damage, showed severe, persistent, pure representational neglect but no evidence of perceptual neglect and no deficits in spatial working memory when evaluated with a traditional clinical test (Corsi Block Tapping test). This finding provides evidence against a full explanation of representational neglect within the context of visuo-spatial working memory. Indeed, this patient showed a peculiar deficit in navigational tasks requiring re-orientation in a novel environment by means of his mental representation of the environment. Since no representational neglect was observed in tests requiring mental representation of single or multiple objects (i.e., o'clock test) we suggest that in our patient representational neglect is caused by damage to the cognitive system involved in coding and storing environmental information to be used during navigation.