A 5-year-old boy with Mietens' syndrome had bilateral microsclerocornea, hypoplastic nose, bilaterally absent radii, elbow flexion contractures, absent left fibula, growth retardation, and normal intelligence. Both corneas measured 9.25 mm in diameter and showed diffuse anterior stromal opacification with focal nebular densities and extensive superficial vascularization. Penetrating keratoplasty in one eye remained clear and compact two years postoperatively and visual acuity improved. Histopathologically, vascularized collagenous tissue occupied the anterior one fourth of the corneal stroma and contained bundles of collagen fibrils 75 to 90 nm in diameter. Descemet's membrane showed abnormal anterior lamination.