A rare case of retrobulbar optic neuropathy caused by an isolated muco
cele in an Onodi cell is presented. A 63-year-old man suffering from l
eft recurrent optic neuropathy had shown improved visual acuity with s
teroid treatment at the age of 48 and 56 years. Impaired visual acuity
and blurred vision in the left eye appeared again, and a marked infer
ior decrease in the left visual field was noted. Although axial comput
ed tomography (CT) showed no abnormal findings, magnetic resonance ima
ging (MRI) revealed a small squamous-shaped lesion(17 x 17 x 7.3 mm) i
n the left posterior ethmoidal air cell (Onodi cell) hanging over the
left optic nerve. Endoscopic sinus surgery was performed. The optic ca
nal was extruding into the Onodi cell cavity, and the superior bony wa
ll of the canal was eroded by the mucocele. The isolated mucocele in t
he Onodi cell seemed to be compressing the optic nerve downward and ca
using retrobulbar optic neuropathy. An Onodi cell lesion, even if it w
as isolated and small, would cause optic neuropathy. MRI and coronal C
T scanning should be performed for the diagnosis and preoperative plan
ning in a case of retrobulbar optic neuropathy.