Primary congenital glaucoma is a potentially blinding disease of children, which if untreated, would result in a lifetime of blindness. It occurs due to obstruction of the drainage of the aqueous humor caused by a primary developmental anomaly at the angle of the anterior chamber. The onset of the disease is in the neonatal or infantile period, and is manifested by symptoms of raised intraocular pressure and corneal edema, such as excessive tearing, photophobia, and an enlargement of the globe (buphthalmos). The consequences of persistently raised intraocular pressure on the optic nerve are far more serious, manifesting as axonal damage and eventual irreversible blindness. Although primary congenital glaucoma is the most common glaucoma seen in infancy, it is still an uncommon disease. The variable incidence in various ethnic groups points towards a genetic basis for the disease. Most cases of primary congenital glaucoma occur sporadically, but in approximately 10% of cases, an autosomal recessive hereditary pattern is evident.