A 15-year-old boy with oculomotor palsy in the left eye as the initial finding of neurofibromatosis type 2 (NF2) was presented. The eye motility examination revealed restriction in adduction, elevation and depression of the left eye and mild ptosis of the left eye. Cranial magnetic resonance imaging (MRI) showed a 3 cm sized mass in the cerebellar vermis that showed features of a schwannoma; bilateral lesions in the acoustic canals and bilateral optic nerve buckling: these findings were consistent with a diagnosis of NF2. Further thin-section MRI revealed a mass in the left cavernous sinus segment of the oculomotor nerve and a thickening of the right oculomotor nerve. © 2010 Informa Healthcare USA, Inc.