Congenital ectropion uveae (CEU) is a rare, non progressive anomaly characterised by the presence of iris pigment epithelium on the anterior surface, an anterior iris insertion, dysgenesis of the drainage angle and glaucoma. The condition is characteristically unilateral and rarely hereditary. The current theory of development arrest is discussed. We report three cases with CEU of which two already developed glaucoma. All patients with CEU should be carefully examined periodically to detect glaucoma.