Glaucoma is a leading cause of blindness worldwide. Normal-tension glaucoma (NTG) is a type of open-angle glaucoma with intraocular pressure measurements always 21 mm Hg or less. A controversy surrounding NTG is the question of whether it should be regarded as a disease within the spectrum of primary open-angle glaucoma or as a distinctive disease entity. Nonetheless, NTG does have distinctive features compared with primary open-angle glaucoma: intraocular pressure-independent risk factors for development of NTG, characteristic patterns of structural and functional damage, and a unique disease course. This review provides an overview and update on the current issues surrounding the prevalence, etiology, diagnosis, and monitoring of NTG.