PAX6 gene (Paired box homeotic gene 6), 11p13, a paired box transcription factor, was described the among the wide set of genes responsible for autosomal dominant congenital cataract (ADCC) [14] that is expressed in the developing eye, brain, spinal cord and pancreas and seems to play a crucial role in the developing of the central nervous system by transcriptional regulation of various target genes [15,16]. This evidence concerns the gene PAX6 and adrenocortical carcinoma, hereditary.