The first description of exfoliation syndrome (XFS) was presented by John Gustaf Lindberg in his doctoral thesis 100 years ago. The syndrome is an age-related disorder in which abnormal fibrillar extracellular material is produced and accumulates in several ocular tissues. Once thought to be peculiar to Scandinavia, XFS is found in almost every race and ethnic group. Subsequent studies provided evidence for the systemic nature of XFS, which involves an aberrant connective tissue metabolism throughout the body. There is a prominent association with zonular instability. This study presents the developments in the understanding of the pathomechanism and clinical significance of XFS and explains the problem of terminology in the description of XFS and pseudoexfoliation.