A missing lens-congenital aphakia?

Congenital aphakia (CA) is a rare disease of the absence of the lens since birth. It may be primary or secondary. Primary aphakia is due to the failure of induction of surface ectoderm to form the lens. It is often associated with ocular maldevelopment. The disappearance of a developed lens by reabsorption or extrusion results in secondary aphakia. A 27-year-old, male patient presented with diminished vision in the left eye since birth, no history of in-utero infection, trauma. On ocular examination, the right eye was normal. The left eye had 30° exotropia; vision was 1/60 with microcornea, corneal opacity, corneal thinning, aphakia, and disc coloboma. B scan revealed posterior staphyloma and aphakia. Congenital Aphakia with associated ocular maldevelopment is a rare case of congenital primary aphakia. It requires a meticulous evaluation and high suspicion to diagnose such a case at birth and follow a multimodal approach to avoid amblyopia and ocular morbidity.