The purpose of our work is to describe the actual knowledge concerning etiopathogenesis, clinical manifestations, diagnostic procedures, complications and therapy of ocular sarcoidosis (OS). The study is based on a recent literature review and on the experience of our tertiary referral center. Data were retrospectively analyzed from the electronic medical records of 235 patients (461 eyes) suffering from a biopsy-proven ocular sarcoidosis. Middle-aged females presenting bilateral ocular involvement are mainly affected; eye involvement at onset is present in one-third of subjects. Uveitis subtype presentation ranges widely among different studies: panuveitis and multiple chorioretinal granulomas, retinal segmental vasculitis, intermediate uveitis and vitreitis, anterior uveitis with granulomatous mutton-fat keratic precipitates, iris nodules, and synechiae are the main ocular features. The most important complications are cataract, glaucoma, cystoid macular edema (CME), and epiretinal membrane. Therapy is based on the disease localization and the severity of systemic or ocular involvement. Local, intravitreal, or systemic steroids are the mainstay of treatment; refractory or partially responsive disease has to be treated with conventional and biologic immunosuppressants. In conclusion, we summarize the current knowledge and assessment of ophthalmological inflammatory manifestations (mainly uveitis) of OS, which permit an early diagnostic assay and a prompt treatment.