Congenital ectropion uveae is a rare disorder resulting from proliferation of iris pigment epithelium on the anterior surface of iris. We describe a case of a 28-year-old female presenting with diminution of vision in the left eye. She was using topical drugs for high intraocular pressure. On examination, visual acuity was 20/60. There was mild ptosis present along with ectropion uveae, 360 degrees around the pupillary border. Intraocular pressure was 32 mm Hg. On fundoscopy, there was glaucomatous cupping with superior notching of the disc. Gonioscopy revealed an open angle with anterior insertion of iris over trabecular meshwork. Visual field analysis showed inferior arcuate scotoma. Examination of the right eye revealed no abnormality and visual acuity was 20/20. No systemic illness was found. The fall in IOP in the left eye following medical treatment remained unsatisfactory. Hence, trabeculectomy was performed. Following a period of 3 months post trabeculectomy, control of IOP was achieved (15 mm Hg). It is important to evaluate for glaucoma in congenital ectropion uveae cases to prevent blindness in young patients and improve their prognosis.