OBJECTIVE: To investigate the clinical features of ocular lesions in Behçet's disease in different countries. METHODS: A descriptive questionnaire survey was performed. RESULTS: 25 eye centres in 14 countries returned questionnaires on prevalent cases in 2006. Clinical data were analysed on 1,465 patients with ocular lesions. Recurrent oral aphthous ulcers were reported in 94.5%, skin lesions in 69.5% and genital ulcers in 61.4%. Most of the patients had bilateral and recurrent intraocular inflammation. Poor visual acuity was seen in 18.9% in women, but 24.8% in men (p<0.01). Panuveitis was seen more in men than in women (p<0.01). 23% of the patients had visual acuity equal to or worse than 20/200 at the final visit. The patients with poor vision were more frequently in India, Iran and Japan than in other countries (p<0.01). CONCLUSIONS: We report the largest contemporary international case series of patients with ocular involvement in Behçet's disease. Panuveitis was significantly more frequent in men than women, and men tended to have a worse visual prognosis. There were some differences in the clinical pattern of Behçet's disease in different countries. Despite modern treatment, the disease still carries a poor visual prognosis with one-quarter of the patients blind.