Background: To determine whether significant compression of the optic nerve by the internal carotid artery (ICA) can produce an optic neuropathy with optic disc cupping that resembles glaucoma in patients without elevated intraocular pressure (IOP). Methods: This was a retrospective case series of patients referred to neuro-ophthalmology for a possible nonglaucomatous optic neuropathy. Patients were included in the study if they had preserved visual acuity, optic discrelated visual field defects, optic nerve cupping, IOP less than 21 mm Hg, open angles, and unequivocal radiological compression of the ipsilateral optic nerve by an intracranial blood vessel. Results: Three patients were included with a mean age of 56.3 (range 29-82) years. Patient 1 was a 58-year-old man incidentally noted to have left optic nerve cupping on a routine examination. He had an inferior arcuate defect and the left prechiasmatic optic nerve was elevated and compressed by a tortuous left ICA. Patient 2 was a 29-year-old man with a normal-tension glaucoma (NTG) diagnosis for 7 years in the right eye treated with latanoprost. He had a superior greater than inferior arcuate defect and there was vascular compression of the optic nerve between the supraclinoid ICA and A1 segment of the anterior cerebral artery. Patient 3 was an 82-year-old woman with an NTG diagnosis for 10 years who had progression of her visual field defects despite low IOPs. MRI showed mass effect on the right optic nerve by a dolichoectatic right supraclinoid ICA. Conclusions: Significant compression of the optic nerve by a normal, tortuous, or dolichoectatic ICA may result in an optic neuropathy with optic disc cupping that resembles glaucoma.