Anophthalmos and microphthalmos are major congenital structural ocular malformations that occur as a result of insults to the developing eye during the first 8 weeks of gestation. Anophthalmos is the congenital absence of the globe, Microphthalmos a condition in which the affected eye is smaller than 15 mm in greatest diameter at birth, less than 19 mm at 1 year or 22 mm in adulthood. Anophthalmos has three distinct types, primary and secondary, degenerative. Anophthalmos is most commonly bilateral, sometimes occurring on one side and microphthalmos on the opposite side. Microphthalmos is of three type’s simple, associated with other anomalies, or with cyst. Anophthalmos/microphthalmos may be due to genetic or non-genetic factors that cause the disease to occur in isolation or as a part of a syndrome. Management of exophthalmos is eyelid surgery or orbital surgery. Recently introduced treatment for microphthalmos is cosmetic scleral shells with optical correction. Other common anterior segment anomalies are anterior segment dysgenesis (ASD), posterior embryotoxon, Axenfeld-Rieger syndrome (ARS), Peter’s anomaly, sclerocornea.