This 36 year-old man was diagnosed first as having glaucoma in 1978. He has had intermittent care throughout his life and has been lost to follow-up many times. He first presented here with no light perception vision in the right eye and 20/40 vision in the left eye. His intraocular pressures were 26 mmHg OD and 13 mmHg OS. He has systemic features of Axenfeld Rieger Syndrome including microdontia and maxillary hypoplasia.