Purpose:To report a case of choroidal neovascularization (CNV) in a patient with cone dystrophy (CD).Methods:Case report.Results:A 20-year-old woman presented with diminished vision in her right eye. Fundus examination showed perifoveal retinal pigment epithelial changes and retinal hemorrhage consistent with subretinal CNV in the right eye, and mild retinal pigment epithelial changes with a dull foveal reflex in the left eye. Optical coherence tomography analysis and fundus fluorescein angiography also confirmed the subfoveal CNV in the right eye. Electroretinography showed decreased amplitudes in photopic and 30-Hz flicker tests in both eyes, which confirmed cone dystrophy. A single intravitreal ranibizumab injection resolved the edema and stabilized the CNV during the follow-up of 6 months.Conclusion:Cone dystrophy is an inherited ocular disorder characterized by loss of cone photoreceptors. Association of CNV has been reported in patients with fundus flavimaculatus, best dystrophy, gyrate atrophy, choroideremia, retinitis pigmentosa, adult-onset foveomacular vitelliform dystrophy, Sorsby macular dystrophy, Bietti crystalline dystrophy, and myotonic dystrophy-related macular dystrophy. We report a case of a patient with CD in whom CNV developed in one eye and responded to a single ranibizumab injection.