Congenital hereditary endothelial dystrophy is characterized by a diffuse bilateral corneal opacity (edema). Its inheritance has been reported to be both autosomal recessive and dominant. All our cases seemed to be autosomal recessive. There seems to be a prevalence of the recessive gene in the Iranian population. The dystrophy might be misdiagnosed as congenital glaucoma, as in several of our cases. We have operated on 37 eyes of 21 patients during the past 10 years. Our visual and anatomical success rate has been very good, with 92% clear grafts and only an 8% rejection rate, in contrast to poor prognosis that has been previously reported. In children, suture removal should be started 3 months postoperatively. Partial amblyopia was preexistent in all cases, but deep amblyopia was not common.