(Pro)renin receptor (PRR) also termed as ATPase H+ transporting accessory protein 2 (ATP6AP2) belongs to type I transmembrane receptor and is capable of binding and activating prorenin and renin. Apart from its association with the renin-angiotensin system (RAS), PRR has been implicated in diverse developmental, physiological, and pathophysiological processes. Within the kidney, PRR is predominantly expressed in the distal nephron, particularly the intercalated cell (IC) and activation of renal PRR contributes to renal injury in various rodent models of chronic kidney disease (CKD). Moreover, recent evidence demonstrates that PRR is primarily cleaved by site-1 protease (S1P) to produce the 28 kDa soluble PRR (sPRR). sPRR seems to mediate most of the known pathophysiological functions of renal PRR through modulating the activity of the intrarenal RAS and provoking pro-inflammatory and pro-fibrotic responses. Not only does sPRR activate renin, it also directly binds and activates angiotensin type 1 receptor (AT1R). The current review will summarize recent advances in understanding the roles and mechanisms of sPRR in the context of renal pathophysiology.