Succinate dehydrogenase (SDH) is an enzyme multifaceted of eukaryotes mitochondria which is reason for converting succinate to fumarate as a component in the cycle of Krebs. Its dysfunction results inside several malignancies related to the endocrine and epithelial tumors. SDH is an enzymatic complex made of some subunits. Succinate is recognized as an oncometabolite so; the discovery of SDH mutations can give a straight connection among the changes of succinate and tumorigenesis. Progresses in laboratory technologies made it possible to make profiles and identification of the accumulation of succinate in several cancer types. Here at this point, we are going to review the potential roles of SDH mutation and changing of succinate in tumorigenesis and as tumor markers for the early discovery of malignancies.