Tumors of the oculomotor nerve are rare and most instances reported have been schwannomas. The authors present clinical, neuroradiological, and neuropathological findings in a 70-year-old woman with a glioblastoma multiforme (GBM) growing primarily in the proximal part of the left oculomotor nerve. The patient presented with a 1-month history of transient diplopia. Neurological examination revealed an incomplete left-sided oculomotor nerve palsy with no further signs of neurological dysfunction. Cranial computerized tomography and magnetic resonance imaging showed a tumor of the left oculomotor nerve without any obvious signs of penetration into the midbrain or upper pons. Following subtotal removal of the tumor, neuropathological examination of the operative specimen revealed a GBM that had grown diffusely within peripheral nerve tissue. Six weeks after surgery, the patient suddenly died of pulmonary thromboembolism. Postmortem examination of the brain confirmed a large leptomeningeal GBM at the left pontomesencephalic junction with complete destruction of the left oculomotor nerve. To the authors' knowledge, this represents the first case of a GBM of the oculomotor nerve, probably originating from glial cells within the most proximal part of the nerve or the adjacent leptomeninges.