The development of congenital non-attachment of the retina is part of the spectrum of the congenital cystic eye in which the stage of the optic vesicle persists. It is caused by the failure of the invaginating anterior optic cup to contact the posterior layer leaving a fluid filled space but the pathogenesis of the non-attachment is not clear .

Complete or partial retinal non-attachment or detachment with retinal dysgenesis, may be associated with many syndromes with abnormal chromosomes and maternal infections and toxins. It may be found in eyes with congenital glaucoma, microphthalmos, persistent hyperplastic primary vitreous, coloboma, and retinal dysgenesis.

Any case of retinal non-attachment may have secondary changes in the anterior segment, in particular shallowing and closed angle glaucoma.