Adreno-cortical carcinoma(ACC)is an uncommon tumor,some of them may produce excessive steroid hormone.The prognosis of ACC is quite poor and current treatment options are relatively limited,especially for pa-tients whose tumor is metastatic and difficult to remove by surgical operation.This makes the management of ACC challenging.Existing studies have shown that the mechanisms of ACC are complex.The molecular changes happen in adrenocortical development and homeostasis may explain the occurrence of ACC.This review focuses on recent studies and summarizes the genetic and molecular mechanism changes that potentially associated with ACC in the development process of the adrenal cortex and multi-omics studies,including genomic changes,epigenetic changes,differences in non-coding RNA,immune microenvironment and features found through multi-omics analy-sis.This information may support screening of therapeutic targets of ACC in future.