Neuroblastoma (NB) is a common pediatric extracranial solid tumor that exhibits varied characteristics, clinical features, and prognosis.1 Totally 1%–2% of cases show familial history with genetic links like ALK, PHOX2B mutations, and 1p36 or 11q14-23 locus deletions. Gastrointestinal stromal tumors (GISTs) are rare mesenchymal neoplasms of the gastrointestinal tract. Approximately 85% of pediatric patients with GIST lack oncogenic mutations in cKIT or platelet-derived growth factor receptor alpha (PDGFRA), and a majority of these are characterized by molecular alterations in the succinate dehydrogenase (SDH) family of genes.2,3 Genetic changes, germline mutations, and variant-phenotype links in NB and GIST remain largely unexplored. Here, we reported a case of biallelic SDHA variant and copy number deletion causing pediatric recurrent NB with GIST to enhance the understanding of this rare clinical scenario.