The authors describe the case of a 42-year-old woman with a 3-year history of recurrent oral and genital ulceration, optic neuritis, and uveitis. She presented with left sided loss of vision. On neurological examination and perimetric analysis a left superior homonymous quadrantanopia was revealed. Magnetic resonance imaging of the brain demonstrated a typical lesion located at the right lateral geniculate nucleus. One month after systemic corticosteroid treatment, her perimetric analysis showed mild improvement. This is a rare case of neuro-Behcet disease presented with an isolated right lateral geniculate lesion.