The intrarenal renin-angiotensin system contributes to the progression of chronic kidney disease. Angiotensin-converting enzyme-2 is a recently identified protein that is highly expressed in the kidney and results in formation of angiotensin-(1-7). This manuscript highlights current information on the localization and function of angiotensin-converting enzyme-2 in the kidney, along with recent studies on the role of the enzyme in hypertension, glomerulosclerosis, and diabetic nephropathy.