Eyes with pseudoexfoliation syndrome (PEX) frequently show clinical signs of impairment of the blood-aqueous barrier. Herein we give an overview of recent studies that analyzed the blood-aqueous barrier in eyes with PEX. METHODS The authors review and summarize recent studies including quantification of aqueous flare in eyes with PEX using the laser flare cell meter (LFCM; Kowa FC-1000) in comparison with normal eyes and eyes with primary open-angle glaucoma (POAG), quantification of aqueous flare in eyes with PEX with and without secondary open-angle glaucoma (SOAG), and quantitative biochemical determination of total aqueous protein concentration in PEX eyes. In addition, studies of noninvasive quantification of the blood-aqueous barrier breakdown following trabeculectomy and following phacoemulsification with intraocular lens implantation in eyes with and without PEX are reviewed. RESULTS In eyes with manifest PEX, both aqueous flare and aqueous protein concentration were significantly increased in comparison with normal control eyes and eyes with POAG. Flare values in PEX eyes with SOAG were not significantly different from flare values in PEX eyes without SOAG. Following trabeculectomy as well as following cataract surgery, breakdown of the blood-aqueous barrier as determined by quantification of aqueous flare was significantly higher in eyes with PEX than in eyes without PEX. CONCLUSIONS Impairment of the blood-aqueous barrier with increase in aqueous protein concentration is a feature of PEX and may be quantified both by flare measurement and by biochemical protein determination. The extensive blood-aqueous barrier breakdown in eyes with PEX following intraocular surgery is an important risk factor for early or late postoperative complications. The alterations of the blood-aqueous barrier should be considered in the medical and surgical treatment of eyes with PEX.