Majority of the pediatric solid neoplasms are sporadic. However, as the understanding of the human genome has increased, a greater number of childhood malignant tumors associated with germline gene mutations or early somatic mosaicism in cancer predisposing genes and well-known clinical syndromes have been recognized. These neoplasms with genetic predisposition constitute up to 10% of all pediatric neoplasms [1, 2]. Cancer predisposition syndromes (CPS) epitomize a rare section of inherited diseases in which children with a pertinent family history or recognized pathogenic germline gene variant are at greater risk of developing malignant and benign neoplasms during their life span compared with their age-matched peers [3]. In certain instances, clinicians may become cognizant of the related CPS through the distinctive phenotypic presentation or, in other cases there may be a positive family history or positive genetic testing. Nonetheless, majority of CPS patients are recognized with the diagnosis of malignancy as the first sign of the genetic mutation with further detection of CPS-related sequelae [2].