According to the Endocrine Society and European Society of Endocrinology, it is recommended that all head and neck paraganglioma patients undergo screening for SDHx and SDHB pathogenic variants since 40% of head and neck PGL patients have germline mutation and 25% of patients with SDHB pathogenic variants have metastasis [13,20,21,22,23,24,25,26]. This evidence concerns the gene SDHB and head and neck paraganglioma.