A rare case of retrobulbar optic neuropathy caused by an isolated mucocele in an Onodi cell is presented. A 63-year-old man suffering from left recurrent optic neuropathy had shown improved visual acuity with steroid treatment at the age of 48 and 56 years. Impaired visual acuity and blurred vision in the left eye appeared again, and a marked inferior decrease in the left visual field was noted. Although axial computed tomography (CT) showed no abnormal findings, magnetic resonance imaging (MRI) revealed a small squamous-shaped lesion (17 x 17 x 7.3 mm) in the left posterior ethmoidal air cell (Onodi cell) hanging over the left optic nerve. Endoscopic sinus surgery was performed. The optic canal was extruding into the Onodi cell cavity, and the superior bony wall of the canal was eroded by the mucocele. The isolated mucocele in the Onodi cell seemed to be compressing the optic nerve downward an causing retrobulbar optic neuropathy. An Onodi cell lesion, even if it was isolated and small, would cause optic neuropathy. MRI and coronal CT scanning should be performed for the diagnosis and preoperative planning in a case of retrobulbar optic neuropathy.