Abstract: Introduction: There is considerable controversy in the characteristics of Behect's disease (BD) in children. The aim of this study is to show Iran's view m juvenile BD. Materials & Methods: Different manifestations of the disease were determined in the BD patients under the age of 16. They were compared with the adult patients by chi square test. Results: We had 102 children with BD. The male/female ratio was 1.04. Oral aphthosis (64%) and uveitis (28%) were the most common presenting symptoms. The prevalence of clinical signs were: Mucosal lesions 85% (oral aphthosis 83%, genital aphthosts 30%); skin lesions 58% (pseudofolliculitis 52%. erythema nodosum 7%); ocular lesions 72%. (ant. uveitis 57%, post. uveitis 63%, retinal vasculitis 39%); articular manifestations 28%; neurologic manifestations 4%;vascular involvement 2% (deep vein thrombosis 1%, superficial phlebitis 1%); gastrointestinal manifestations 5%; orchi-epididymitis in 8% of boys. There was no ankylosing spondylitts (AS) or cardiopulmonary involvement. In paraclinical tests: Pathergy test was positive in 56%. HLA-B5 in 50%, HLA-B27 in 20%. Abnormal urine sediment was seen in 8%. Comparison of these data with those of adults showed these lesions significantly less in children: Mucosal lesions both oral and genital (p<0.000001), skin lesions (p<0.0002) both pseudofolliculitis (p<0.002) and erythema nodosum (p=0.00006), joint manifestations (p<0.005). phlebitis (p<0.02) and hematuria (p<0.04). White ocular signs (p<0.008) and positive HLAB27 (p<0.002) was seen more. Conclusion: Juvenile BD differs from adults. Oral aphthosis is not prevalent enough, at least to be used as a mandatory criterion for diagnosis. The nature of some manifestations differs (such as articular and skin lesions), while some are not present at all (such as AS). In Iran ocular lesions, either at the onset or during the course of the disease, are one of the most prominent signs in juvenile BD, with a potential cause of blindness just as JCA.