Two 6-month-old white boys developed localized multifocal white nodules of the anterior corneal stroma without evidence of antecedent trauma, inflammatory disease, or familial occurrence. The first patient had bilateral lesions initially; the second patient initially had unilateral involvement followed by a corneal nodule in the second eye at 11 years of age. Light and electron microscopic studies of the lesions in both patients were similar and disclosed stromal nodules composed of proliferating myofibroblasts, activated fibroblasts, and haphazardly arranged fascicles of collagen. Immunohistochemical studies revealed that the spindle cells within the nodules expressed immunoreactivity for vimentin and alpha-smooth muscle actin. Management options for these slowly progressive corneal lesions include lamellar versus penetrating keratoplasty and topical corticosteroids.