Penetrating keratoplasty in infants and young children is performed on an infrequent basis. The most common indication is visually significant congenital corneal opacity. Surgery must be performed early to avoid amblyopia. Surgical techniques differ from those used in adult penetrating keratoplasty because of the reduced ocular rigidity encountered in infants and young children. Use of a multispecialty team approach is important to improve visual outcome. Poor prognostic indicators include bilateral disease, concomitant infantile glaucoma, lensectomy and vitrectomy at the time of surgery, previous graft failure, extensive goniosynechiae, and extensive corneal vascularization. Prompt postoperative optical rehabilitation, combined with occlusion therapy when appropriate, is an important determinant of success.