The paper presents the clinical characteristics of Behcet's disease (BD) in Russian children, generally accepted criteria for BD, and diagnosis statement. The investigation is based on an observation of 17 children who met the international criteria for BD and were treated at the V.A. Nasonova Research Institute of Rheumatology in 2004 to 2013. One-third of children had a compromised family history; about 30% of the patients were ethnic Russian. The age at BD onset ranged from infancy to 16 years. The interval between disease onset and diagnosis was 1 to 10 years. The clinical symptoms at onset represented mostly frequently aphthous stomatitis and its accompanying fever and much less frequently aphthae of the genitalia. Furtherthe pathological process involved other organs and systems. In advanced stage of disease, there was already a clinical syndrome encompassing aphthous stomatitis, a genital ulcerative process, and lesions of the eye, skin, gastrointestinal tract, and less often central nervous system. The manifestations of the disease in different combinations can be concurrent with each other, showing a mosaic pattern. Treatment of children with BD included glucocorticoids, colchicine, azathioprine, and, if clinically indicated, tumor necrosis factor-α inhibitors. Their efficacy is discussed. The pediatric aspects of BD, which are presented in this paper, are first published in Russian medical press