Familial aniridia is an autosomal dominant condition in which only a small iris remnant is present. A controversy exists as to whether the pathogenetic, embryologic origin of this condition is neuroectodermal or mesodermal. We examined a male infant with bilateral congenital aniridia associated with persistent pupillary membranes. This child had a prominent arcade of vessels extending onto the anterior lens surface for 360 degrees from small iris remnants in both eyes. The presence of a mesodermal pupillary membrane despite absence of the iris suggested a primary neuroectodermal defect as the pathogenetic factor in this case of aniridia.