Children with some genetic syndromes are predisposed to developing Wilms tumor. These include WAGR , Denys Drash syndrome (renal failure, renal mesangial sclerosis, male hermaphroditism, WTl missense mutation) and Beckwith-Wiedemann syndrome (hemihyperophy, macroglossia, omphalocele, organomegaly, del llp15.5-WT2). Opsoclonus-myoclonus-ataxia syndrome is associated with neuroblastoma Opsclonus-myoclonus syndrome is a rare paraneoplastic disorder, often associated with malignancies including of childhood derived from the