Acquired syphilis uveitis, due to lack of the characteristic features, presents with various types. The most common type is posterior uveitis and panuveitis, including chorioretinitis, retinal vasculitis, optic nerve retinitis. The diagnosis and assessment of response to treatment depends mainly on the serological diagnostic tests, including nontreponemal and treponemal test. Acquired syphilis uveitis often presents with manifestations similar to various types of uveitis, especially to autoimmune uveitis and other infectious uveitis, so differential diagnosis is important. The gold standard treatment for active syphilitic uveitis is penicillin G, or doxycycline if patient is allergy to penicillin. Clinically misdiagnosis and delayed treatment may result in irreversible visual impairment and severe systemic and eye complications. However such timely treatment always has a good prognosis.