Primary infantile glaucoma, commonly termed congenital glaucoma or trabeculodysgenesis, is an unusual, inherited connatal anomaly of the trabecular meshwork and anterior chamber angle which leads to obstruction of aqueous outflow, increased intraocular pressure, and optic nerve damage. Its pathogenesis is still disputed; most observers have not been able to document ultrastructurally a continuous endothelial membrane, as initially advanced by Barkan. Medical therapy for primary infantile glaucoma is accorded a supportive role; the primary, definitive treatment is surgical. Both goniotomy and trabeculotomy ab externo give similarly good results in the majority of patients. The prognosis in this disease is related to the time of its initial presentation, initial surgical intervention, degree of optic nerve damage, nature and quality of corneal enlargement and astigmatism, progressive refractive error, and anisometropic amblyopia. The inability to easily quantitate visual acuity and extent of visual loss in neonates makes these parameters less helpful in following patients than measurement of corneal diameter and intraocular pressure. However, even these data should not be relied upon exclusively to determine the quality or quantity of success in primary infantile glaucoma.