Pseudoexfoliation syndrome is an age-related systemic disease that mainly affects the anterior structures of the eye. Despite a worldwide distribution, reported incidence and prevalence of this syndrome vary widely between ethnicities and geographical areas. The exfoliative material is composed mainly of abnormal cross-linked fibrils that accumulate progressively in some organs such as the heart, blood vessels, lungs or meninges, and particularly in the anterior structures of the eye. The exact pathophysiological process still remains unclear but the association of genetic and environmental factors are thought to play a role in the development and progressive extracellular accumulation of exfoliative material. Hence, LOXL1 gene polymorphisms, responsible for metabolism of some components of elastic fibers and extracellular matrix, and increased natural exposure to ambient ultraviolet or caffeine consumption have been associated with pseudoexfoliation syndrome. Ophthalmological manifestations are commonly bilateral with an asymmetric presentation and can lead to severe visual impairment and blindness more frequently than in the general population, mainly related to glaucoma and cataract. Pseudoexfoliation glaucoma is a major complication of pseudoexfoliation syndrome and represents the main cause of identifiable glaucoma worldwide. Visual field progression is more rapid than that observed in primary open angle glaucoma, and filtering surgery is more frequently required. Nuclear cataract is more frequent and occurs earlier than in the general population. Owing to poorer pupil dilation and increased zonular instability, cataract surgery with pseudoexfoliation is associated with a 5- to 10-fold increase in surgical complications compared to cataract surgery without pseudoexfoliation. Some specific treatments targeting production, formation or accumulation of exfoliative material could improve the prognosis of this syndrome.