Purpose: To present a rare case report of Onodi cell-associated optic neuropathy, conducting a review of the literature.Methods: A 36-year-old male presented with an 18-h history of acute deterioration of vision in his left eye (LE). Ophthalmic examination and Magnetic Resonance Imaging (MRI) were consistent with an Onodi cell-associated compressive optic neuropathy.Results: Despite immediate, successful surgical decompression, severe optic nerve atrophy and permanent visual loss occurred during early postoperative period. The reported case gives rise to different hypotheses regarding pathophysiology that may lead to irreversible blindness. A systematic review of the respective literature is provided attempting to compare different approaches in the management of Onodi cell-associated compressive optic neuropathy and assess their efficacy in the final visual outcome. Poor initial visual acuity (VA) may represent a bad prognostic factor. Moreover, age and gender do not seem to significantly influence the outcome.Conclusion: This report and associated literature review highlight the importance of the radiologic characteristics and early diagnosis in the final visual outcome of the Onodi cell-associated optic neuropathy. High level of suspicion is crucial for early diagnosis of mucoceles, which must be treated promptly by surgical and medical means to enhance visual recovery.