According to the latest report, 31.5% of the adrenal oncocytoma are related to hormone abnormalities, including hypercortisolism and sex hormone abnormalities.[8] The most common clinical manifestations are Cushing's syndrome, masculine and feminine.[9] Another system review showed that only 17% are functional adrenal masses, and an adrenocortical oncocytic neoplasm occurring with a Cushing's syndrome, pheochromocytoma, or aldosteronoma had been described.[10] In this study, the youngest patient is only 5 years old, and 2.3%(3/11) patients had the functional tumors. This evidence concerns the gene PLXNA3 and adrenal gland hyperfunction.