Adrenocorticotropic hormoneindependent macronodular adrenal hyperplasia (AIMAH) is a rare adrenal pathological lesion. AIMAH presents clinically as overt or subclinical Cushing's syndrome with typical radiological findings, occasionally with other diseases. What is rarer is the familial type. The mechanism of AIMAH may be the aberrant expression of several hormones and cytokines receptors in adrenal, which leads to the oversecretion of glucocorticoid, and the molecular mechanism involves genic mutation or deficiency of the receptors. Although presently adrenalectomy is the main treatment, with further research on pathophysiology，more and more effective medication will be used. The research progress of clinical features, mechanism, and treatment of AIMAH is reviewed in this paper.