Abstract Chronic kidney disease is a leading cause of mortality and morbidity in Western countries and is estimated to affect 11% of the adult population. The possibility of treatment of chronic kidney disease has been severely impaired by our poor knowledge of the regenerative properties of the kidney. Recent results obtained in humans, together with genetic tagging experiments performed in rodents, demonstrated that the epithelial components of the cortical nephron share a unique progenitor, which can generate podocytes as well as tubular cells. Accordingly, lineage tracing experiments demonstrated that bone marrow-derived interstitial or papillary cells are not involved in the repair of injured adult renal epithelium. In addition, assessment of the markers CD24 and CD133 in adult human kidney as well as genetic tagging in rodents allowed us to identify a hierarchical population of renal progenitors arranged in a precise sequence within Bowman's capsule. The results of all of these studies suggest that the kidney contains a “renopoietic system,” with a progenitor localized at the urinary pole of Bowman's capsule, from where it can initiate the replacement and regeneration of glomerular, as well as tubular, epithelial cells. Knowledge of renal progenitor cell biology may enable a better comprehension of the mechanisms of renal repair as well as more effective targeted therapies for acute and chronic kidney diseases. Disclosure of potential conflicts of interest is found at the end of this article.