This chapter provides a concise and practical review of the main clinical characteristics of hereditary syndromes associated with pheochromocytoma/paraganglioma (PPGL). Neurofibromatosis type 1 (NF1), von Hippel Lindau syndrome (VHL), and multiple endocrine neoplasia type 2 (MEN2) are complex syndromes with extremely variable phenotype, whose clinical course is only partially governed by PPGL development. Also mutations in the FH gene, which have only recently been associated with PPGL predisposition may express a complex and variable phenotype; on the contrary, PPGL is the most common and characteristic expression of SDHx mutations, and more rarely mutations in these genes may also predispose to renal cancer and gastrointestinal stromal tumors. Identification of an inherited PPGL has important implications for both the patients and their relatives, who will need to be offered genetic screening. Patients with PPGL hereditary syndrome and asymptomatic carriers of mutations in PPGL susceptibility genes need an extensive and lifelong surveillance program which considers their risk of relapse or development of new PPGL and the risk related to the involvement of other organs.