We will review the 2007/2008 literature on pediatric genitourinary tumors. Newly identified constitutional epigenetic defects in Wilms tumor genes extend the understanding of Wilms tumor risk in children lacking syndromic features, and add to the complexity of the pathogenesis of these tumor suppressor genes. Pediatric renal cell carcinoma has distinct molecular characteristics and clinical associations from the adult counterpart. The pathway from PAX3-FKHR translocation to the development of rhabdomyosarcoma tumors has been further elucidated. Therapeutic strategies continue to be driven by developments in molecular diagnostics in pediatric genitourinary tumors.