A 52-year-old woman presented with pain, decreased visual acuity, decreased color vision, and visual field loss in the OD. Fundus examination revealed optic disc edema and later a macular star figure consistent with neuroretinitis. Magnetic resonance imaging showed enhancement of the orbital optic nerve sheath and perineural fat suggestive of optic perineuritis. The patient was not treated. At 11 months, visual function and fundus examination had returned to normal. This unusual simultaneous occurrence of neuroretinitis and optic perineuritis suggests that these two entities may have a similar pathogenesis.