Topographical disorientation is usually described in patients who have lost the ability to orient themselves as a consequence of acquired focal brain damage. Here, we describe the case of a 20-year-old woman with a congenital brain malformation who has never been able to orient herself within the environment. We addressed in detail her ability to orient and navigate within the environment by administering a number of tasks in both ecological and experimental surroundings. The results indicate a complete inability to use any kind of strategy useful for orientation.