A congenital neoplasm of the inner layer of the anterior lip of the optic cup is delineated. Because of continued growth, local excision was performed at 6 months of age, followed by cardiac arrest. The neoplasm demonstrates features predominantly of a glioneuroma, but with transitions to a teratoid medullo-epithelioma. Local invasion of iris, ciliary body, trabecular meshwork, retina, and sclera was apparent. An associated finding was persistence of the primary vitreous and hyaloid artery in the opposite eye.