Ocular involvement in Behçet disease occurs is most patients and can be the presenting organ. Intra-ocular inflammation (uveitis) related to Behçet disease is mainly a panuveitis including an occlusive retinal vasculitis. Recurrent inflammation can result in the development of ocular complications, including macular edema and retinal neovascularization, with up to a quarter of eyes developing vision loss. Diagnosis is based on clinical ophthalmic findings and ocular imaging including retinal fluorescein angiography and optical coherence tomography. Early diagnosis and treatment may prevent the development of ocular complications and vision loss. Treatment is based primarily on systemic corticosteroids and 2nd-line immunosuppressive agents, particularly anti-tumor necrosis factor α agents. Extensive treatment, prevention of relapses and complications can result in long term stable vision.