Normal tension glaucoma (NTG) has similar optic neuropathy as primary open-angle glaucoma (POAG), but intraocular pressure (IOP) is within the normal range. Compared with high-pressure POAG, the development of NTG is possibly a consequence of a complex interaction of several ocular and systemic factors. Recent data have shown higher translaminar pressure gradient due to impaired cerebrospinal fluid dynamics may account for the pathogenic mechanism. Insufficient blood supply and vascular dysregulation may also be the cause of the disease. In clinical evaluation, NTG is a diagnosis by excluding other nonglaucomatous optic neuropathies. NTG is characterized by larger and deeper optic-disc cupping, more central visual field defects and a higher incidence of disc hemorrhage compared with POAG. In clinical practice, controlling IOP as low as possible (with medication, laser trabeculoplasty, or surgery) is the key to manage NTG patients. In addition to IOP reduction, the control of systemic risk factors or improving ocular perfusion may be beneficial therapies. NTG is more prevalent in Asia than in the Western countries. Due to increasing old population and underdiagnoses in the clinical settings, NTG became a great challenge for ophthalmologist in Asia. Therefore, the aim of this article is to focus on the epidemiology, to update pathogenesis, diagnosis, and management for NTG.