Even if the risk of developing a tumour during childhood (<18 years old) is low, a small number of SDHB-related paragangliomas have been reported at age 6 years13,20,30–35 and children with an SDHB mutation have a higher risk of developing a metastatic paraganglioma than children with mutations in the other SDHx genes32,36. The gene discussed is SDHB; the disease is paraganglioma.