A 19 years boy with a 2 years history of reduced and fluctuating vision along with change in pupillary shape and iris colour in his left eye presented to the glaucoma clinic. Ocular examination revealed distinct unilateral stretch holes, iris architecture changes and localized iris atrophy. Intraocular pressure was 16 mmHg in the right and 36 mmHg in the left eye. Gonioscopy of the left angle revealed broad based peripheral anterior synechiae at approximately 2 O'clock to 10 O'clock hours. The optic disc of the left eye had a vertical cup of 0.9 which manifest as superior and inferior arcuate scotoma. Specular microscopy showed unilateral abnormal endothelium with irregular cells of variable shape and size. To our knowledge, this is only the second reported case of iridocorneal endothelial syndrome in a male teenager.