There are several genetic disorders associated with pediatric cancers. The occurrence of bilateral, multifocal, or metachronous neoplasms and specific histopathologic findings can suggest a genetic predisposition syndrome.  Constitutional pathogenic variants can now be routinely screened by next generation sequencing (NGS) to identify at-risk individuals in affected families and offer genetic counseling, choice of therapy and surveillance guideline. ¹  Recent pediatric genomic germline sequencing studies reported that at least 10% of pediatric cancer patients, with or without a family history, carry a mutation in a known cancer predisposition gene, ² including previously unrecognized associations.  ³ See details in 5th edition (2022) WHO Peadiatric Cancers  Syndromes associated with specific organ systems (digestive, endocrine system, breast, CNS, female genital tract, skin, bone and soft tissue) can be found in the corresponding volumes of the WHO Classification of Tumours.