Behçet's disease (BD) is a multisystem immune-mediated inflammatory disorder. The choice of treatments depends on organ involvements and its severity. The treatments for pediatric patients usually consist of corticosteroids and immunosuppressants, but some of them are refractory to these treatments. Recently, tumor necrosis factor alpha (TNF-alpha) appears to play an important role in the immunopathogenesis in BD. We describe two patients with childhood-onset BD, successfully treated by anti-TNF agents. One patient, a 18-year-old male, was diagnosed as having BD at 8 years of age. He developed abdominal pain and bloody diarrhea at 17 years of age. Gastrointestinal endoscopic examination revealed esophageal and ileocolic ulceration consistent with intestinal BD. Methylpredonisolone pulse therapy and immunosuppressants were administrated, but did not respond. Infliximab infusions (4 mg/kg) were given at weeks of 0, 2, 6, and then every 8 weeks. A dramatic improvement in gastrointestinal symptoms and extraintestinal manifestations were observed. Another patient, a 9-year-old girl, developed recurrent oral ulcers and folliculitis at 4 years of age. She was diagnosed as having BD at 6 years of age, and treated with colchicines, corticosteroids and immunosuppressive agents. She was suffering from fever, malaise, and arthralgia in addition to mucocutaneous manifestation. At 8 years of age, etanercept was started at a dosage of 25 mg subcutaneously twice a week. A resolution of the systemic and mucocutaneous symptoms was achieved. Furthermore, she was free from corticosteroids in three months. Anti-TNF therapy would be a good choice for juvenile BD refractory to conventional treatments.