Ong et al. (2007) evaluated the genotype–phenotype correlations in 573 VHL patients and confirmed that pheochromocytoma was linked to VHL missense mutations. Additionally, the age at onset for VHL syndrome was significantly earlier (P = 0.001) and the age-related risks of RA and RCC were higher (P = 0.022 and P = 0.0008, respectively) for individuals with nonsense or frameshift mutations compared to those with deletions. Here, VHL is linked to pheochromocytoma.