Intraneural perineurioma is a true but rare neoplasm that originates from perineurial cells and mainly affects peripheral nerves. It must be distinguished from other hypertrophic neuropathies that are either inflammatory or demonstrate an onion-bulb formation that originates from Schwann cells. Complying with this strict definition, only three additional cases of cranium-related perineurioma have been identified: two lesions arose extracranially and involved cranial nerves, and one occurred intracranially but did not involve a nerve. The authors describe a 27-year-old woman who presented with left third cranial nerve palsy and was found to harbor a mass lesion in the superior orbital fissure and cavernous sinus. After subtotal resection had been performed, pathological studies confirmed the presence of perineurial tumor cells in a pseudo-onion bulb formation. The cells stained positively for epithelial membrane antigen but not for S100 protein, clearly distinguishing the disease from one that originates in Schwann cells.