The efficacy of corneal transplantation in infants with corneal opacity secondary to congenital glaucoma has not been established. We retrospectively reviewed our results of nine penetrating keratoplasties performed on eight eyes of six infants who had multiple risk factors for poor prognosis: age <2 years at the time of grafting; uncontrolled glaucoma in four eyes; concurrent lensectomy, retinal, or glaucoma surgery in five eyes; aphakia in five eyes; and an acute perforation in one eye. Six of the nine grafts (67%) remained clear during a mean follow-up of 24 months (30 months in eyes with clear grafts). Development of ambulatory vision or better occurred in six of eight (75%) eyes after corneal transplantation and treatment of refractive errors and amblyopia. Graft failure occurred in three eyes—two from corneal decompensation, and one from homograft rejection. Complications included one total retinal detachment, one case of Streptococcus pneumoniae keratitis, and three cases that lost intraocular pressure control, requiring further glaucoma surgery. We conclude that useful vision can be achieved after penetrating keratoplasty even in some high-risk infants with congenital glaucoma.