The authors present a general review of autosomal dominant transmission of polycystic kidneys. It represents 10% of all causes for haemodialysis. Its penetrance is close to 100%. The dominant aetiology at the present time is obstruction due to hyperplasia of the tubular epithelium which induces cystic dilatation which extends to involve all of the nephron. The cysts are cortical and medullary. The diagnosis is based on an association of enlarged kidney and a family history. The commonest associated abnormalities are hepatic cysts in 60% of cases, cerebral aneurysms in 10 to 20% of cases and colonic diverticulosis in 80% of cases. At the stage of renal failure, patients must be treated for hypertension and deterioration in nephron function must be prevented by a low protein diet. When the patient reaches the stage of renal failure and must be dialysed, he must be rapidly enrolled in a transplantation programme as the actuarial graft survival is more than 80% at 1 year and more than 70% at 5 years.