Unilateral, spontaneous, non-pigmented iris cysts appeared before the age of 2 years in four patients. Histopathological specimens obtained in three cases showed stratified to cuboidal, non-pigmented, epithelial lined cysts. Goblet cells were recognised in two of the three specimens. The clinical features and histopathological findings indicate that these cysts are derived from surface ectoderm and may be congenital.