Pheochromocytoma is a tumor derived from chromaffin tissue in the adrenergic system with excessive secretion of catecholamine.Pheochromocytoma occurs at any age of patients，commonly in 40-60 years，and the incidence is slightly higher in women than in men.In recent years，studies have shown that the mutations of von Hippel-Lindau gene (VHL)，rearranged during transfaction gene (RET)，neurofibromatosis type 1 gene (NF-1)，succinate dehydrogenase gene (SDH)，transmembrane protein 127 gene (TMEM127)，myelocytomatosis oncogene-associated factor X gene (MAX) are associated with pheochromocytoma.Immunohistochemical studies have revealed that a number of molecular markers，such as telomerase，vascular endothelial growth factor，cyclooxygenase-2，adrenomedullin，plasma chromaffin protein A，signal transducer and activator of transcription-3 are of velue in identification of tumor origin，its biological behaviors and differentiation of pheochromocytoma. This article reviews the newest research progresses in molecular biology of pheochromocytoma.