Behçet’s disease (BD) is a form of systemic vasculitis with the highest prevalence in the Mediterranean area and the Far East, and is very rare in Poland. The syndrome is a multisystem disorder characterized by recurrent oral and genital ulcers, relapsing uveitis, articular, neurological, vascular and pulmonary manifestations. Although the usual onset of the disease occurs after puberty, and between the second and fourth decades, there has been increased awareness of patients with onset before puberty. The aim of our study is to evaluate the clinical spectrum in two patients in whom the disease was fully manifested and diagnosed during childhood. Two cases of childhood BD from Poland are presented in the paper. Based on the reported cases diagnostic and therapeutic problems of BD are discussed. Our results point out to a similar systemic expression of BD in children and adults. Behçet syndrome may be more common in the children than previously reported.