Congenital iris ectropion is an uncommon malformation and no reports exist about the use of modern technologies in this pathology. The authors describe a case of unilateral and isolated congenital iris ectropion associated with juvenile glaucoma in a healthy and completely asymptomatic 6-year-old girl with an unusual form of anisocoria. Optical coherence tomography and confocal scanning laser tomography showed a progressive glaucomatous neuropathy. A trabeculectomy without antimetabolites was performed and intraocular pressure normalized without other medications during a follow-up of 2 years. The authors assert the utility of various diagnostic technologies to recognize congenital iris ectropion early to prevent blindness in young patients and improve their prognosis.