We present nine patients with unilateral congenital ectropion uveae (hyperplasia of the iris pigment border), iris hypoplasia, characteristic gonioscopic findings, and glaucoma. A majority of these patients also demonstrated mild ptosis. In none of these patients were there any associated systemic abnormalities. The apparent ectropion uveae results from spread of the iris pigment epithelium beyond the iris ruff and onto the anterior surface of the iris. In two patients with this abnormality, histopathologic and electron microscopic findings are presented. All patients with congenital ectropion uveae should be carefully examined periodically to detect glaucoma.