Purpose: To report the results of long-term topical treatment with bevacizumab (Avastin) 5 mg/mL eyedrops in a case of aniridia-related neovacularization of the cornea. Methods: Interventional case report. A female patient with aniridia had a decrease in the best corrected visual acuity from 0.32 to 0.02 in the OS over the course of 4 years, secondary to central corneal neovascularization and epithelial breakdown. Vision in the OD was 0.2. In 2008, at age 28, a shared decision was made to start off-label treatment with bevacizumab eyedrops 0.5% in both eyes. After 9 years, the visual acuity in the OD remained stable, with stability of the macropannus and maintenance of central corneal clarity. In the OS, the central corneal neovascularization regressed, the epithelium regained its clarity, and after cataract surgery visual acuity was regained to 0.32. Results: After 9 years of treatment with topical bevacizumab, vision acuity is comparable to the situation of 12 years previously: Visual acuity remained stable in the ODS. Conclusion: In a young patient with progressive corneal neovascularization secondary to aniridia, stability of central corneal neovascularization was obtained and corneal clarity was preserved by adding a daily drop of bevacizumab 5 mg/mL. No adverse events occurred. Vessel growth was inhibited, and as such, the progression of the natural history of the patient’s disease was halted. More clinical study with longer follow-up is needed to investigate the applicability of treatment with topical VEGF inhibitors in aniridia.