The clinical and pathologic findings of a case of congenital bilateral, peripheral corneal leukoma associated with iris coloboma and anterior subluxation of the lens are reported. The changes in the region of keratolenticular apposition include partial absence of Bowman's membrane, stromal vascularization and thinning with large-diameter collagen fibrils, and absence of Descemet's membrane and endothelium. The authors postulate that dysembryogenesis of the tertiary vitreous, with iris coloboma, resulted in apposition of the lens to cornea during the second and third month of gestation and that this apposition resulted in the observed peripheral corneal abnormalities.