Behçet's disease is a vasculitis that can cause inflammatory lesions in multiple organs or systems including the nervous system. Most cases worldwide have been reported along what is called the "Silk Route" from the Mediterranean region to Japan, so it is considered a rare disease in Latin American countries. The frequency of neurological involvement ranges from 5 to 13%. We present the case of a young adult woman with diagnostic criteria for Behçet's disease and manifestations of neurological disease, as well as a review of the literature.