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Posner-Schlossman syndrome (PSS) or glaucomatocyclitic crisis is a rare, unilateral recurrent inflammatory ocular hypertensive disease.Although it is typically self-limited and has benign prognosis, some cases were reported with advanced optic nerve cupping and associated visual field loss.Current therapeutic strategies are mainly focused on controlling intraocular pressure and reducing inflammation.These treatments may relieve acute episode of PSS, but could not decrease the recurrences.Therefore, the etiology of PSS is extremely important.This review summarized and analyzed the advances in the etiology and pathogenesis of PSS in recent twenty years, including microbial infection, allergy, abnormal vascular reactivity, autoimmune and endocrine.Infectious theories are most studied at present.The probabilities of varicella-zoster virus and herpes simplex virus as the etiology of PSS were considered to be very small.Heliobacter pylori was thought to be related to the pathogenesis of PSS and glaucomatous optic nerve injury.The most likely cause of PSS is cytomegalovirus infection, but there existed evidences that opposed the theory.Due to the small sample size and limit of technology, the evidences of allergy, abnormal vascular reactivity, autoimmune and endocrinic factors as the pathogenesis of PSS were not definite.In conclusion, no single factor could explain the etiology and pathogenesis of PSS, multiple factors such as external factor, internal factor and autogenous factor might be involved.