Central retinal vein occlusion (CRVO) is frequent in patients with systemic lupus erythematosus (SLE), but the treatment of the macular edema with this disease is extremely difficult. We report a case of cystoid macular edema (CME) secondary to unilateral CRVO in a patient with SLE that responded to intravitreous injection of an anti-vascular endothelial growth factor (VEGF) agent. A 33-year-old Japanese woman was referred to our department with unilateral impairment of vision. Microperimetry (MP-1) showed a cessation of foveal sensitivity. Fluorescein angiography showed CME without ischaemia of the macular region or peripheral retina (nonischemic CRVO). A diagnosis of CME and unilateral nonischemic CRVO combined with SLE was made and intravitreous anti-VEGF therapy was given. A sample of aqueous humor was harvested at the start of intravitreous injection after obtaining informed consent. Then the levels of VEGF and monocyte chemotactic protein (MCP)-1 were measured in the aqueous humor by enzyme-linked immunosorbent assay, revealing that VEGF was 234 pg/mL and MCP-1 was 501 pg/mL. Two weeks later, left eye vision improved to 20/20. Optical coherence tomography (OCT) showed considerable amelioration of retinal swelling and CME. MP-1 showed a marked increase of foveal sensitivity. However, she had recurrence of edema 3 months later. After harvesting aqueous humor again, intravitreous injection of an anti-VEGF agent was repeated for CME. The aqueous VEGF and MCP-1 levels were 156 pg/mL and 360 pg/mL, respectively. These findings suggest that inflammation was improved by intravitreous injection of bevacizumab. Intravitreous injection of anti-VEGF agents may be effective for CME due to nonischemic CRVO in SLE patients if their inflammatory factor levels are low.