This case report aims to describe a case of unilateral central retinal artery occlusion (CRAO) with cilioretinal artery sparing, which was complicated by neovascular glaucoma (NVG). A 75-year-old Indian woman with underlying normal tension glaucoma presented with the sudden onset of painless generalized blurring of the right eye's vision for a week. Her right eye vision was hand motion with the presence of a right relative afferent pupillary defect. Fundus examination revealed retinal whitening over the macula sparing the papillomacular bundle with generalized retinal arteriolar attenuation, which was suggestive of right CRAO with cilioretinal artery sparing. Systemic examination revealed high blood pressure (175/75 mmHg) without ocular bruit or audible murmur on auscultation. Optical coherence tomography of the macula showed inner retinal thickening over the temporal macula. Ultrasound carotid Doppler and computed tomography angiography of the carotid showed more than 75% stenosis over the right distal internal carotid artery. Unfortunately, she developed rubeosis iridis over her right eye two weeks after her presentation, which required pan-retinal photocoagulation. She subsequently progressed to NVG, requiring maximum anti-glaucoma medications to stabilize intraocular pressure. In conclusion, CRAO is a sight-threatening medical emergency. Thorough investigations are required to determine the underlying cause so that early intervention can be done to reduce the risk of a similar attack in the fellow eye and the risk of a cerebrovascular event or cardiac ischemia, which could be life-threatening. The presence of a cilioretinal artery does not prevent ocular neovascularization in CRAO. Hence, patients should also be closely monitored after the initial diagnosis to prevent devastating complications such as NVG.