A retrospective review of 29 patients with neurovascular compression syndrome (NVCS) involving the anterior visual pathway was conducted. Various patterns of NVCS and visual defects were identified, most commonly involving the optic nerve and internal carotid artery. Most patients were stable, except one with progressive visual field defects. Although mostly asymptomatic, NVCS can rarely cause compressive optic neuropathy. NVCS should be kept in the differential diagnosis of normal tension glaucoma, especially with progressive visual loss despite treatment. Patients with progressive visual loss may require decompression surgery. Non-contrast computed tomography scan may miss NVCS, and magnetic resonance imaging is diagnostic.