The incidence of syphilis and syphilitic uveitis in our community is increasing. The prevalence of associated neurosyphilis is unknown, and it remains unclear whether syphilitic uveitis should be treated as secondary syphilis with intramuscular penicillin or neurosyphilis with intravenous penicillin. The (English language) literature was reviewed for all unique cases of syphilitic uveitis reported from 1984 to June 2008. For each case the following data were recorded: the clinical features of the syphilis, the uveitis and any associated neurosyphilis, the human immunodeficiency virus (HIV) status, lumbar puncture findings, treatment and follow up. We identified 143 patients in 41 original reports of syphilitic uveitis (93 HIV-positive and 50 HIV-negative). Posterior uveitis was reported in 79 patients (55.2%); panuveitis was reported in 36 patients (25.2%); anterior/intermediate uveitis was reported in only 28 patients (19.6%). Lumbar puncture findings were abnormal in 82 patients (57%), and the majority of these patients (76%, 62 out of 82) were HIV-positive. One hundred and ten (77%) patients were treated with intravenous therapy, usually penicillin. Most recovered from the syphilis, however, a proportion did not recover full vision. There were 13 (9%) treatment failures, which tended to occur in patients who were HIV-positive (n = 11), had abnormal lumbar puncture findings (n = 8) and/or were treated (n = 11) intravenously. There is a high incidence of abnormal lumbar puncture findings in patients with syphilitic uveitis and a strong association with HIV infection. Most received appropriate therapy with a low relapse rate, which was not related to the type of therapy.