To report both the efficacy and safety of interferon-alpha-2a (IFN-alpha) therapy in corticodependent uveitis of paediatric Behçet's disease (BD). Data from seven children affected with corticodependent uveitis of BD and treated with IFN-alpha were reviewed retrospectively. IFN-alpha was injected sub-cutaneously thrice a week at dosages of 1.5-3 M(ons) IU according to the children's weight. Efficacy was judged on the ability of IFN-alpha to induce a corticosteroid (CS)-sparing effect while maintaining remission. All adverse events (AE) were recorded. The children included four boys and three girls. Mean age at onset of uveitis was 8.6 yrs and mean follow-up duration was 7.14 yrs. All children had a high level of corticodependence and five of them received additional DMARDs. A remarkable CS-sparing effect with remission maintenance was achieved in 5 out of 7 patients after a mean period of 14.6 months of IFN-alpha administration. The remission was sustained in four of the five patients (mean = 4.8 yrs), even after IFN-alpha was discontinued in three of them. The other patient relapsed 1.5 yrs after IFN-alpha discontinuation. The last two patients faced early severe adverse events attributed to IFN-alpha: retinal venous thrombosis and major depression. IFN-alpha has a potent CS-sparing effect in paediatric BD patients suffering from severe uveitis. However, the possibility of major side-effects with this treatment calls for careful monitoring.