Postoperative vision loss following spine surgery is a rare and devastating complication with variable incidence. Various risk factors have been identified in the literature. A 16-year-old male presented with neck pain, spastic quadriparesis, and tingling paraesthesia in the left upper limb for the past 3 years. Radiological workup was suggestive of atlantoaxial dislocation with occipitalization of the posterior arch of the atlas (C1). The patient was prone to a horseshoe headrest and underwent occipito-C2-C3 fixation by removing the posterior rim of foramen magnum. Postoperatively, the patient complained of loss of vision in the right eye. Ophthalmology evaluation revealed a loss of perception of light in the right eye due to central retinal artery occlusion. An injectable steroid was started, but there was no improvement in vision in the involved eye. Proper head positioning and avoidance of intraoperative hypotension are modifiable factors to avoid such devastating complications.