We preliminary examined genotype–phenotype correlation in VHL families and our data support the previously distinction described in the literature between VHL types 1 and 2 regarding missense variants and their association with a higher risk of pheochromocytoma (Crossey et al., 1994; Maher et al., 1991; Zbar et al., 1996); 80% of our tested individuals with PHEO presented a missense variant. This evidence concerns the gene VHL and hereditary pheochromocytoma-paraganglioma.