Two cases of unilateral ectropion uveae with ipsilateral juvenile glaucoma are reported including one with ipsilateral ptosis. This rare, non progressive anomaly, is characterized by the presence of iris pigment epithelium on the anterior surface of iris stroma, quite often associated with neurofibromatosis, ipsilateral ptosis or Rieger's syndrome. Congenital or juvenile glaucoma is almost systematically associated with the condition and must be evaluated and treated. Pathogeny of the anomaly has to be interpreted keeping in mind the concept of neurocristopathy, i.e. developmental anomalies of neural crest origin tissues.
