Abstract: Pheochromocytomas and paragangliomas (PPGLs) deriving from chromaffin cells in the adrenal medulla or extra-adrenal sympathetic or parasympathetic system are rare neuroendocrine tumors. More than one third of these tumors are identified to be related with germline gene mutations that may increase the distinct differences in tumor metabolism and directly affect the patients' clinical manifestations and follow-up management. Therefore, PPGL genomics was closely related with the biological behaviors of tumors, imaging features, genetic screening, and patient management. This review will summarize the new developments and significance of PPGL genomics.