To describe the multimodal imaging findings and treatment outcomes in choroidal neovascularization secondary to Choroideremia. A 13-year-old male presented with reduced visual acuity in the left eye. He gave a history of nyctalopia. His best-corrected visual acuity (BCVA) was 20/20 in the right eye and 20/40 in the left eye. Based on multimodal imaging, the diagnosis of choroideremia in both eyes with a subfoveal choroidal neovascularization in the left eye was made. He underwent five intravitreal anti-vascular endothelial growth factor (VEGF) injections of Ranibizumab over a period of 3 years, with the final injection given due to recurrence of neovascularization. Post-treatment, his BCVA improved to 20/20 in the left eye with regression of the neovascular network. This case highlights the role of OCTA in diagnosis of choroidal neovascularization in choroideremia as well as its successful management with anti-VEGF injections with long term follow up. © 2021 The Authors.