Congenital glaucoma results from a failure of the normal development of the tissues at the angle of the anterior chamber, which impedes the outflow of aqueous humour from the eye.

In primary congenital or infantile glaucoma, blockage results from an arrest in development of the angle structures at about the seventh month of foetal life.

In other congenital glaucomas there may be an association with other congenital anomalies such as aniridia, Sturge Weber, Marfan's syndrome and neurofibromatosis.