Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of kidney failure. Scientific advances have improved the approach to diagnosis, prognosis, and management of renal and extrarenal manifestations. The combination of total kidney volume, kidney function, and the genetic mutation (if known), predicts risk for progression to kidney failure, thereby identifying patients in whom disease modifying therapy is recommended. Currently there is one therapy approved by the US Food and Drug Administration (FDA) for slowing ADPKD progression, the V2 receptor antagonist, tolvaptan.