There has been a great deal of clinical work done on the management of MTC in MEN2. In particular there have been several trials of tyrosine kinase inhibitors which have shown efficacy. In contrast, there have been only case reports of the use of these agents in PC. Sunitinib is reported to have some activity but other agents have not been systematically studied. Pheochromocytomas usually present in the third or fourth decade of life in patients with MEN2A. Generally the diagnosis is confirmed at the same time as or following the diagnosis of medullary thyroid carcinoma (MTC). Suspicion of the presence of MEN2 and in particular for pheochromocytoma is sometimes provided by the pattern of catecholamine (and catecholamine metabolite) excretion with raised epinephrine or epinephrine and norepinephrine being characteristic. Genetic testing is recommended to identify a heterozygous germline RET pathogenic mutation. Since 1993 and the identification of RET as the causative gene of multiple endocrine neoplasia type 2, genotype/ phenotype correlations have identified mutations likely to be associated with pheochromocytoma and with age specific disease penetrance of these mutations, as discussed in the revised ATA Guidelines1. Recent understanding of pheochromocytoma development includes the role of hypoxic and RET pathways, which may indicate a role for tyrosine kinase inhibitors. Trials of these agents should be undertaken and will require international collaboration.