Pseudoexfoliation syndrome (XFS) is a condition in which excess material builds up not only in the structures of the anterior chamber but also throughout the body. The frequency of the syndrome varies significantly (0.3–18%) depending on the region and the method of examination. Environmental risk factors for XFS include a large number of sunny days, proximity to the equator, dietary factors such as higher consumption of coffee and tea, long-term alcohol consumption, exposure to UV, and outdoor work. The pathognomonic sign of XFS is the presence of white material on the lens capsule and other anterior chamber components. In addition, a characteristic Sampaolesi line can be observed during gonioscopy. Systemic alterations indicative of XFS have been observed in the extracellular matrix of the eyelid skin, the heart, lungs, liver, kidneys, gallbladder, meninges, and endothelium of the blood vessels. XFS is the most common cause of secondary open-angle glaucoma, which is called pseudoexfoliative glaucoma and is more severe than primary open-angle glaucoma. It is plausible that a combination of environmental factors and genetic alterations promotes the onset of pseudoexfoliation syndrome, which requires additional research.