Iridoschisis is a rare condition that consists of the separation of the anterior mesodermic layer of the iris. It is associated with glaucoma in 50% of cases. The association of this disorder with luetic interstitial keratitis has been reported rarely. We report the case of a 58-year-old patient who had unilateral iridoschisis associated with glaucoma and syphilitic interstitial keratitis. We suggest that congenital syphilis might be considered as a cause of iridoschisis.