Objective To evaluate the clinical characteristics of patients with adrenocorticotropin-independent macronodular adrenal hyperplasia (AIMAH).Methods A total of 10 AIMAH cases were enrolled in this retrospective study.The clinical and laboratory findings of all patients were collected and analyzed.Results All patients manifested some clinical features and biochemical evidence of Cushing's syndrome.The plasma adrenocorticotropic hormone (ACTH) level was undetectable in all the patients and their serum cortisol secretion rhythm was abnormal.Low and high-dose dexamethasone suppression tests failed to suppress the cortisol secretion.The bilateral macronodular adrenal enlargement was shown by CT/magnetic resonance imaging.The supine-upright posture test was positive in four patients.Three patients were performed bilateral adrenalectomy,five were unilateral adrenalectomy and the remaining two patients were taken propranolol.All the patients had followed up for 10 to 89 months.Contralateral adrenalectomy was performed in two patients with recurrent symptoms after unilateral adrenalectomy and two patients given propranolol were underwent bilateral adrenalectomy when their symptoms had not been improved or recurred.Conclusion AIMAH is a relatively rare subtype of Cushing's syndrome with unique clinical and laboratory findings.Propranolol is a good choice if the supine-upright posture test is positive.Unilateral adrenalectomy appears to be an effective and safe alternative treatment for AIMAH.Bilateral adrenalectomy could be performed if the symptoms have not been improved or recurred after unilateral adrenalectomy.