Autosomal dominant polycystic kidney disease is the third most common cause of renal failure with no definitive treatment available that can directly target the development and growth of the cysts. Endeavours are being made to retard the growth of the cysts and preservation of renal function through medical treatment. However, 50% of the autosomal dominant polycystic kidney disease-affected persons develop complications and end-stage renal disease by the age of fifty-five and need surgical intervention for the management of complications, creation of dialysis access and renal transplantation. This review highlights the principles and current practice pertinent to the surgical management of autosomal dominant polycystic kidney disease.