Behçet’s syndrome (BS) causes panuveitis and retinal vasculitis in about 50% of patients. Despite intensive treatment, up to 20% of patients may lose useful vision. 1 Treatment consists of corticosteroids, immunosuppressive agents such as azathioprine (AZA) and ciclosporin A (CycA), 1 and biological agents such as interferon-α (IFN) and antitumour necrosis factor agents. 2 3 We describe a patient with juvenile BS whose refractory eye disease was treated successfully with canakinumab, a fully human anti-interleukin-1β antibody. Figure 1 shows the different treatment regimes used during the follow-up period. A 16-year-old girl was diagnosed with BS at the age of 9 years because of recurrent oral ulcers, erythema nodosum, eye disease and a positive pathergy test. Her initial ophthalmological examination showed features of bilateral panuveitis and retinal vasculitis. Her visual acuity was 0.1 in the right eye and 0.2 in the left eye. She was prescribed AZA 2.5 mg/kg/day and CycA 5 mg/kg/day together with prednisolone. She continued to have severe eye attacks with hypopyon especially in the left eye under this treatment and her visual acuity deteriorated in both eyes to finger counting level. AZA and CycA were stopped 6 months later and IFN was started at a dose of 5 MU/day. While she was on IFN her visual acuity improved (0.9 in the right eye, 0.2 in the left eye). However, she developed fever and erythema nodosum so IFN was stopped after about 5 months and infliximab infusions (5 mg/kg) were started, in addition to the combination of AZA and CycA. She was attack-free for the first 2 years but experienced frequent flares of uveitis. After another 6 months, infliximab was switched to adalimumab. There was an initial response to adalimumab, however her eye disease relapsed after about 12 months. Adalimumab was given for another 20 months, increasing the dose of prednisolone. Her vision had fallen to< 0.1 and optic atropy had developed in the left eye by this time. After adalimumab she was given anakinra, 2 mk/kg, a recombinant human interleukin 1 (IL-1)