Primary hyperaldosteronism resulting from adrenocortical carcinoma is very rare. We have experienced a 39 year old man with adrenocortical csrcinoma presenting with hypertension, hypokalemia, and metabolic alkalosis. The patient had a 13 cm encapsulated mass in his left adrenal gland and multiple metastatic nodules in the lungs. Hyperaldosteronism was established as the cause of the hypertension on the basis of laboratory tests showing suppressed plasma renin activity and nonsuppressible high plasma aldosterone concentration. He has been placed on chemotherapy with cisplatin containing regimen and interferon-a for more than 6 months. He is in stable condition with no evidence of progress in the lung and recurrence in the adrenal gland. A review of the literature revealed only 8 cases with primary hyperaldosteronism resulting from pure aldosterone excess produced by adrenocortical carcinoma. In Korea, oui report is the first one showing clear histologic findings and endocrine tests.