Primary aldosteronism (PA) is a typical example of low renin hypertension, whereas renal artery stenosis (RAS) is a classic form of high renin hypertension. PA and RAS occurring simultaneously in a patient is challenging to diagnose. We report a 32-year-old woman with a 12-year history of resistant hypertension. She was identified to have elevated plasma aldosterone and renin levels with normal aldosterone/renin ratio (ARR). Imaging examinations identified bilateral adrenal thickening and subtotal occlusion of the anterior segment of the left renal artery. Adrenal venous sampling was performed and indicated the existence of unilateral aldosterone over-secretion. It may suggest that even though RAS led to non-suppressed renin, adrenal venous sampling remains to be an applicable approach to establish the diagnosis of aldosterone-producing adenomas, although the diagnostic value of ARR may be compromised due to non-suppressed renin level. The patient underwent a two-stage treatment. First, stenosis of the left renal artery was dilated by percutaneous transluminal renal balloon angioplasty. Two months later, laparoscopic complete left adrenalectomy was performed. Hematoxylin-eosin staining and CYP11B2 immunostaining suggested that this tumor was an aldosterone-producing adenoma. After the two-stage treatment, her blood pressure decreased to a normal level without antihypertensive drugs. This case report raises our awareness of the simultaneous occurrence of RAS and PA. Under this condition, ARR could lead to a false-negative PA. Adrenal venous sampling is warranted to achieve a confirmed diagnosis. For subjects with complex etiologies of secondary hypertension, multi-stage treatment may be required.