PURPOSE: To present a case with co-existence of Cogan-Reese syndrome and exfoliation syndrome.CASE REPORT: A 72-year-old Caucasian woman presented for consultation due to uncontrolled intraocular pressure (IOP) of the right eye. Clinical examination revealed the presence of Cogan-Reese syndrome and exfoliation syndrome OD. This eye exhibited elevated IOP, open anterior chamber angle, advanced glaucomatous optic nerve head damage, and severe glaucomatous visual field loss. The left eye was completely normal without IOP elevation or visual field damage. To our knowledge this is the first case report demonstrating the coexistence of the Cogan-Reese syndrome, exfoliation syndrome, and secondary open-angle glaucoma. Since both syndromes frequently lead to secondary open-angle glaucoma, it is not feasible to determine with certainty which condition was the cause of secondary open-angle glaucoma in our case. It is conceivable that both conditions contributed to glaucoma development.CONCLUSIONS: Cogan-Reese syndrome, exfoliation syndrome and secondary open-angle glaucoma may present on the same eye.