Syphilis is a sexually transmitted, chronic, systemic infection caused by the spirochete Treponema pallidum. If left untreated the disease progresses through 3 stages. The ocular manifestations are highly variable, including interstitial keratitis, anterior, intermediate and posterior uveitis, chorioretinitis, retinitis, retinal vasculitis and optic neuropathy which can occur at all stages. In patients with a suspected diagnosis it is mandatory to carry out serological tests, because an early treatment is essential for preserving visual acuity and ocular function.