Autosomal dominant polycystic kidney disease is a common (approximately 1 in 400 individuals in the United States) inherited disorder, in which hypertension is the most often associated disorder. Although the development of hypertension originates with expansion of renal cysts, it most likely has its pathogenesis in the renal vasculature. Evidence is now accumulating that the renin-angiotensin-aldosterone system is important in the development and maintenance of hypertension in this disorder. End-organ damage including left ventricular hypertrophy and progressive renal insufficiency appear to be related to the presence of hypertension in ADPKD. A focus on early detection and adequate control of hypertension in ADPKD, therefore, may be associated with improved cardiovascular and renal outcomes as well as increased patient survival in ADPKD.