A 48-year-old woman complained of acute loss of vision in her right eye. Ophthalmoscopically, the right optic disk appeared pale, and abnormally dilated vessels were noted on the disk. The left optic disk was reddish and swollen. Fluorescein angiography revealed abnormal vessels on the right optic disk that might be venous. The patient had right-sided anosmia. Computed tomography and magnetic resonance imaging showed a lesion in the frontal lobe that was deviated to the right and attached to the olfactory groove. A histopathologic study of the excised specimen disclosed a meningioma. We believe that this patient with olfactory groove meningioma represents a rare case of Foster Kennedy syndrome and optociliary shunt vessels.