A woman in her early 40s with a history of optic disc melanocytoma experienced acute vision loss to hand-motions level secondary to acute hemiretinal vascular occlusion. On subsequent follow-up visits, the vision deteriorated to no light perception and the patient developed optic disc neovascularization refractory to conventional laser therapy, which was complicated by vitreous hemorrhage, preretinal fibrosis, and tractional retinal detachment (Figure). Although melanocytomas are considered benign lesions, severe vision loss can rarely occur secondary to vascular occlusion, or spontaneous tumor necrosis due to the progressive subtle growth of the tumor within the optic nerve.1-3 To our knowledge, this is the first report of ischemic retinopathy with extensive optic disc neovascularization refractory to conventional medical treatment secondary to an optic disc melanocytoma.