Abstract-We have identified 2 relatively young patients with significant hypertension, an elongated single aberrant renal artery supplying blood to a renal segment, and evidence for localization of the elevated plasma renin activity to the side and vein draining the affected kidney. Furosemide-induced diuresis and acute oral captopril stimulated the renal vein/contralateral renin ratios to 4.3:1 and 6.5:1 in patients 1 and 2, respectively. These renal vein ratios are significantly higher than normal (Ͼ3:1 under similar conditions). Partial resection of the portion of the kidney affected by the aberrant tortuous artery led to a marked reduction in blood pressure in patient 1. Patient 2, not an operative candidate, responded satisfactorily to use of a converting enzyme inhibitor, which helped to confirm the dependency of the blood pressure on the abnormal flow relationship existing within that aberrant artery and the kidney. We believe these 2 patients are representative of a small but distinct subgroup within the larger number of patients with elongated single or multiple renal aberrant arteries. Each aberrant artery had no focal stenosis, although a decrease in flow relative to the tissue perfusion demands was apparent from the marked activation of the renin-angiotensin system in the venous system draining that artery. The increased length of such vessels may contribute to their decreased flow, although their average diameter may reside just above such a critical value for a normal length vessel. This new syndrome, involving more than one component of the flow/resistance relationship, has been overlooked when renin-dependent forms of hypertension are considered. Key Words: angiotensin Ⅲ arteries Ⅲ hypertension, renal Ⅲ renin Ⅲ vessels A possible association between accessory renal arteries and hypertension has been suspected over a long time. However, this relationship has received comparatively little attention in terms of its existence because of conflicting or inconclusive data, and many texts do not assign it pathophysiologic significance. It is not surprising that little literature exists as to possible etiologic factors.Accessory renal arteries, like hypertension, are common. Variations of renal arteries are recorded from the midnineteenth century, 1 and the frequency varies from 25% to 61% of cadavers. [2][3][4][5] The association of hypertension with multiple renal arteries first appeared in the 1930s and included anomalies involving the renal pelvis as well. 6 -9 Since 1951, Marshall 10 and other authors reported an increased presence of multiple renal arteries in hypertensive patients compared with normotensive patients. [11][12][13][14][15] Within this population, some had focal stenosis of an aberrant artery and were identified as having a variant of classical renal artery stenosis and hypertension.A persuasive literature supports a dominant role for activation of the renin-angiotensin system in maintaining the elevated systemic pressure in patients with unilateral renal artery stenosis. 16 -20 Rece...