Invasive rhino-orbito-cerebral aspergillosis usually affects immune-compromised state patients such as diabetes. Proptosis, visual loss, and ophthalmoplegia due to intra-orbital extension are common presentations. It is well known that perineural extension is usual in invasive mucormycosis, but only one case of perineural extension was reported in invasive rhino-orbito-cerebral aspergillosis. Recently, we experienced a case of a 63-year-old woman with invasive rhino-orbito-cerebral aspergillosis in right PNS and orbit. She underwent surgical excision of fungal material and decompression of orbit due to subperiosteal abscess and consequent right visual loss. After surgery we treated her with 25 days of antifungal agent medication but visual loss of contralateral eye occurred. Because perineural extension to optic chiasm and contralateral optic nerve was found in brain MRI, left optic canal decompression was done secondarily. Three weeks after the secondary operation, left visual acuity was recovered and no other complication happened after all. We report this case with a review of the related literatures.