Introduction The present study aimed to analyze the clinical features of ocular pathology in patients with acquired immunodeficiency syndrome (AIDS) combined with syphilis. Methods A total of 129 patients with AIDS and syphilis who first visited the Department of Ophthalmology in Beijing YouAn Hospital between 2012 and 2019 were included in the study. All patients underwent ophthalmologic examinations, such as best-corrected visual acuity (BCVA), slit lamp, intraocular pressure, dilated fundus examination, and color fundus photography as well as systemic examinations related to AIDS and syphilis. The patients were divided into four groups according to fundus disease: a normal fundi group, an HIV-related microvascular retinopathy (MVR) group, a cytomegalovirus retinitis (CMVR) group, and a syphilis-related retinopathy group. Results The incidence of fundus disease was 70.7%. There were 36 patients with normal fundi (29.3%), 40 with HIV-related MVR (31.0%), 25 with CMVR (19.4%) (including 11 cases of CMVR with syphilis-related retinopathy), 26 (20.2%) with syphilis-related retinopathy, 1 (0.78%) case with acute retinal necrosis, and 1 (0.78%) case with PORN. The median blood CD4 + T-cell count in the syphilis-associated retinopathy group was 357.5 cells/mu l, which was significantly higher than in the other groups; this difference was statistically significant. In the CMVR group, 11 cases with concomitant syphilis-associated retinopathy had lower BCVA and 10 (90.9%) had active inflammatory manifestations in the anterior segment. Conclusion The incidence of ocular pathology was high in patients co-infected with AIDS and syphilis, which might manifest in a variety of ocular manifestations; some patients may also have multiple ocular changes, which should be given great clinical attention.