WE REPORT the case of a boy who was first seen in our clinic at the age of 4 months because of anisocoria with a mydriatic pupil in his left eye(Figure 1). In the following years, he developed unilateral progressive iris ectropion (Figure 2 and Figure 3).His intraocular pressure and optic disc were normal at the age of 5 years. He was then lost to follow-up and seen 10 years later with an intraocular pressure of 50 mmHg and end-stage glaucoma in his left eye (Figure 4). The development of glaucoma in congenital ectropion uveae after several years after birth strongly implies progression of chamber angle changes after birth. We herein show that iris changes may also progress during infancy.