Pheochromocytomas and rarely paragangliomas occur in 10-50% of patients with VHL syndrome usually around 30 years of age and exceptionally before the age of 10.<h4>Case presentation</h4>We diagnosed a 9-year-old girl of normal appearance and severe refractory hypertension, with a norepinephrine-secreting pheochromocytoma related to VHL syndrome due to a known familial germline heterozygous mutation of <i>VHL</i> gene (c.414A>G), also present in three members of her family. This evidence concerns the gene VHL and pheochromocytoma.