Some sporadic cases have a risk of developing Wilms tumor as a part of WAGR (Wilms tumor, aniridia, genitourinary abnormalities, and mental retardation; OMIM 194072), which is caused by deletion of both PAX6 and Wilms’ tumor gene (WT1) in the 11p13 region. Here, PAX6 is linked to Nephroblastoma.