Many inherited diseases and non-hereditary disorders have in common the development of renal cystic disease. The most common, autosonal dominant polycystic kidney disease, is responsible for 5-10% of end-stage renal failure treated by dialysis or transplantation. Since the publication six years ago of the last book on polycystic kidney disease, a number of genes causing the disease have been identified, mapped, or sequenced; new experimental models and the application of molecular biology techniques have provided new insights into the pathogenesis R polycystic kidney disease; novel clinical studies have provided valuable information for the prevention, evaluation, and treatment of the complications of this disease. This book provides an updated, state-of-the-art review of the genetics, pathophysiology, evaluation, and management of these diseases and will be of interest to both basic researchers and clinicians in nephrology.