Once viewed as a hopelessly incurable disease, autosomal dominant polycystic kidney disease (ADPKD) has been given a great deal of attention as geneticists search for ADPKD genes, and cell biologists are studying and understanding cyst formation. Alterations in cellular growth, secretion, and extracellular matrix all participate in cystogenesis. ADPKD is one of the most common hereditary diseases in the United States and is a frequent cause of chronic renal failure. The disease is a systemic disorder that may affect multiple organ systems. Manifestations of ADPKD in renal and extrarenal systems may be challenging problems in the management of these patients. Although no therapy is available, treatment of the complications is highly effective in most cases in supportive management of the disease.