Pseudoexfoliation syndrome (PEX) is characterized by the deposition of proteinaceous material in the anterior ocular segment (resulting in ophthalmic pathologies such as glaucoma and increased risk of complications in cataract surgery), but also by several systemic manifestations. The involvement of peri-ocular tissues in PEX, including the eyelid skin, lacrimal gland, conjunctiva, orbital fat and vessels, as well as the optic nerve, has been reported by several previous studies. The peri-ocular effects of PEX include the development of eyelid laxity, conjunctival chalasis, tear film abnormalities, pronounced orbital fat atrophy in response to the administration of prostaglandin analogues in pseudoexfoliative glaucoma, deficient orbital vascular supply and biomechanical changes in both the eyeball and the optic nerve. These effects may have important clinical implications, including increased difficulty in cataract surgery, ocular surface disease and eyelid margin malpositions.