Spontaneous congenital and acquired nonpigmented epithelial iris cysts are rare unilateral lesions of the anterior uvea. From 1980 to 1991, eight infants with progressive nonpigmented epithelial iris cysts were successfully treated by block excision. This technique consists of simultaneous removal of the adjacent iris, the pars plicata of the ciliary body, and all layers of the sclera and cornea in contact with the lesion that serve as a shell for the proliferating epithelium. The resulting defect is covered with a tectonic corneoscleral graft. Five of the children, whose ages ranged from 1 month to 7 years (average, 3.7 years), presented with a congenital nonpigmented epithelial iris cyst and three infants presented with a cystic epithelial ingrowth following perforating injury. On histopathological examination, all but one patient revealed epithelial involvement of the surface of the ciliary body. All patients were followed for an average of 45.5 months. The long-term visual acuity of our very young patients was encouraging, ranging from 20/200 to 20/20. No recurrence of iris cysts was noted and no enucleation was necessary. Our results indicate that block excision may be the treatment of choice for congenital and infantile nonpigmented epithelial iris cysts.