Background: Spinal cord involvement in Behçet’s disease is not well studied. Objective: To evaluate the clinical, laboratory and magnetic resonance imaging characteristics of spinal cord involvement in Behçet’s disease. Methods: We retrospectively reviewed 10 spinal cord involvements in seven patients with Behçet’s disease. Results: The median age of onset for spinal cord involvement was 32 (23–45 years). Two patients showed a secondary progressive course. Cerebrospinal fluid findings revealed mild to moderate pleocytosis and/or elevated protein levels. In eight spinal cord involvements, the lesion was longer than three vertebrae. Serum anti-aquaporin-4 antibody was negative in all four patients tested. Conclusions: Longitudinally extensive transverse myelitis is a characteristic manifestation of spinal cord involvement in Behçet’s disease.