Prorenin receptor (PRR), a 350-amino acid receptor initially thought of as a receptor for the binding of renin and prorenin has been shown to be multifunctional. In addition to its role in the renin angiotensin system (RAS), PRR also transduces several intracellular signaling molecules and is a component of the vacuolar H+-ATPase that participates in autophagy. PRR is found in the kidney and particularly in great abundance in the cortical collecting duct. In the kidney, PRR participates in water and salt balance, acid-base balance, autophagy and plays a role in development and progression of hypertension, diabetic retinopathy, and kidney fibrosis. This review highlights the role of PRR in the development and function of the kidney namely the macula densa, podocyte, proximal and distal convoluted tubule and the principal cells of the collecting duct and focuses on PRR function in body fluid volume homeostasis, blood pressure regulation and acid-base balance. This review also explores new advances in the molecular mechanism involving PRR in normal renal health and pathophysiological states.