Contrary to the preoperative expectations of pheochromocytomas, all the tumors from the 3 patients who had undergone adrenalectomy were diagnosed with adrenocortical adenomas, which showed positive for CYP11B1 with AMH in the associated adrenal parenchyma. Here, CYP11B1 is linked to hereditary pheochromocytoma-paraganglioma.