Aniridia (absence of iris) is a congenital, bilateral, uncommon panocular disorder. Whereas the occurrence of aniridia in the general population is 1:50000, it is present in about 1 in 70 patients with Wilm's tumor. This aniridia is sporadic and Wilm's tumor in these cases presents at an unusually early age. Aniridia was present in two cases out of 60 cases of Wilm's tumor operated at PGIMS, Rohtak. Both cases presented below two years of age. The recognition of a child with sporadic aniridia should alert to the increased risk of development of Wilm's tumor.