A 32-year-old patient with recurrent sinusitis had severe visual loss from optic neuropathy. Imaging revealed severe bone destruction and soft tissue densities of the paranasal sinuses and enhancement of the dura of the frontal sinuses, optic canals, and superior orbital fissures bilaterally. Endoscopic sinusectomy with biopsy showed granulomatous vasculitis compatible with Wegener granulomatosis (WG). The patient was treated with intravenous and oral corticosteroids and oral cyclophosphamide that led to rapid and dramatic visual recovery. This case draws attention to the fact that optic neuritis may be an early inflammatory manifestation of WG and that rapid diagnosis and aggressive anti-inflammatory treatment is critical before inflammation of arteries leads to infarction and irreversible visual loss.