Behcet's disease is a systemic vasculitis, characterized by recurrent oral aphthous ulcers, recurrent genital ulcers, skin lesion, and ocular involvement. Monoclonal antibody to the tumor necrosis factor-α (TNF-α) is considered as a possible therapeutic approach to achieve clinical improvement, preventing relapse in Behcet's disease refractory to conventional anti-inflammatory drugs or immunosuppressive durgs. We report the use of infliximab, which is one of the TNF-α monoclonal antibodies, in a 17-year-old girl with Behcet's disease exhibiting severe mucocutaneous, ocular and neurological involvement refractory to standard treatment.