Normal-tension glaucoma (NTG) is a progressive optic neuropathy with intraocular pressure (IOP) within the statistically normal range (≤21 mmHg). The prevalence of NTG varies widely among different population studies, being the most prevalent open-angle glaucoma subtype in some reports. The etiology of NTG possibly is multifactorial and still not well defined. Alternative treatments have been proposed based on pathogenesis details. However, in clinical practice, adequate reduction of IOP remains the keystone of managing patients with NTG. We review the pathogenesis of NTG and the available therapies for this optic neuropathy.