Although neurological involvement in Behçet's disease is not so uncommon, isolated spinal cord disease is quite rare and reported to be observed in about 2% of all cases with neurological involvement. Here we report a Behçet's patient with spinal cord disease presented with anterior spinal cord syndrome. This rare syndrome is caused by hypoperfusion of the anterior spinal artery and to our knowledge has not been previously reported in patients with Behçet's disease. This report defines the characteristic clinical features of this entity and emphasizes the importance of early immunosuppressive treatment and initiation of rehabilitation.