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A 57-year-old woman presented with subacute vision loss of first the left, and later the right eye. She was diagnosed with granulomatous polyangiitis with hypertrophic pachymeningitis and optic nerve compression. Her visual acuity could not be permanently restored with immune suppressants alone, so a surgical decompression of the right optic nerve, via a modified cranio-orbitozygomatic pretemporal approach, was performed. Her right eye regained 20/20 vision and has remained stable during 8 months of follow-up.

Successful surgical optic nerve decompression in a patient with hypertrophic pachymeningitis due to granulomatous polyangiitis