A 14-year-old girl with typical oculodentodigital syndrome had been treated for chronic glaucoma for seven years. She had corneas measuring R.E.: 10 mm; L.E.: 9 mm, with narrow angles open on the right, but almost entirely closed on the left. Gonioscopic examination of the right iris revealed that the root was attached forward on the trabecular meshwork, which suggested a congenital anomaly. Peripheral iridectomies were ineffective. In a case previously studied, a 20-year-old woman with typical oculodentodigital syndrome developed typical acute angle-closure glaucoma seven years after the original examination.