Congenital aphakia is a rare anomaly that can be subdivided into 2 forms: primary and secondary. Histologically, the lens is absent in primary congenital aphakia. The histologic findings of secondary congenital aphakia depend on the underlying etiology. Primary congenital aphakia results from failed induction of the surface ectoderm during embryogenesis and has been associated with mutations in the PAX6 gene and severe ocular and systemic developmental anomalies. In secondary congenital aphakia, the lens has developed but has been resorbed or extruded before or during birth. This form of aphakia is often associated with congenital infections, such as congenital rubella.