A rare case of congenital bilateral ectopia lentis et pupillae (ELEP) is reported in a 32 year old female presenting with complaints of diminution of vision in both eyes. Associations of optic nerve hypoplasia and myelinated nerve fibers are being reported for the first time in such a case. The aetiopathogenesis of ELEP is controversial. Combined neuroectodermal and mesodermal origin is being postulated for this syndrome on the basis of aforesaid abnormalities and presence of pupillary dilator muscle hypoplasia, "annular endotheliopathy", and persistent pupillary membrane in both eyes.