Congenital ectropion uveae (CEU) is a rare disorder characterized by overflows of the iris pigment epithelium covering the anterior iris surface, angle dysgenesis, and progressive glaucoma. The condition is generally nonprogressive but the main complication is congenital or juvenile glaucoma. This anomaly is correlated with neural crest dysplasia, and can be associated with some genetic diseases. The most typical manifestation is the pigment epithelium of the iris overflows into the pupil margin and covers a variable portion of the anterior iris surface. It may cover much of the iris, but does not extend to the iris root. Almost all patients require filtering surgery to control intraocular pressure.