A 34-year-old woman with active granulomatosis with polyangiitis previously receiving cyclophosphamide presented with 2 days of right eye pain, proptosis, and complete vision loss. Her granulomatosis with polyangiitis affected the skin, lung, kidney, and eye, including a known right orbital mass (visual acuity was 20/20 OD). Examination of the right eye demonstrated a visual acuity of no light perception, pale optic disc, retinal whitening without cherry-red spot, arteriolar attenuation with boxcarring, and venous engorgement with associated tortuosity and flame hemorrhages (Figure, A). Magnetic resonance imaging of the brain and orbits revealed severe proptosis from tissue infiltration of the orbit encasing the optic nerve with resultant traction (Figure, B).