Abstract. The present study was performed to clarify changes in plasma levels of 19-hydroxyandrostenedione (19-OH-AD), an amplifier of aldosterone and a possible hypertensinogenic steroid, during several tests for the renin-angiotensin system in 20 patients with aldosterone-producing adenoma (APA) and to determine whether 19-OH-AD participates in the etiology of the hypertension in this disorder. Basal plasma 19-OH-AD levels in patients with APA were significantly lower than those in 50 normal subjects, and correlated positively with basal plasma cortisol levels (r=0.45, P<0.05). Plasma 19-OH-AD levels were not changed significantly by 2-h standing during which plasma renin activity (PRA) remained suppressed. With 40 mg iv furosemide plus 2-h standing during which PRA remained suppressed, plasma 19-OH-AD levels increased significantly with a concomitant significant increase in plasma cortisol. With dexamethasone pretreatment, however, such positive responses of plasma 19-OH-AD and cortisol disappeared. After the removal of the APA with the adjacent adrenal tissue, PRA and plasma aldosterone concentrations became normal or low-normal, but plasma 19-OH-AD and cortisol did not change as compared with the preoperative levels. There were no significant correlations between basal plasma 19-OH-AD levels and mean blood pressure either before or after the adrenal operation. These findings suggested that 1) the secretion of 19-OH-AD in patients with APA is reduced due to the chronically suppressed renin-angiotensin system, 2) but is still concomitantly under the control of the ACTH-adrenal axis and 3) 19-OH-AD may, at least, not play an important causative role in the hypertension commonly observed in patients with APA.