The records of 65 patients with adult type polycystic kidney disease were examined in an attempt to identify the problems and priorities in the management of these patients, with particular reference to ultimate haemodialysis or transplantation. The three main problems of patients presenting before the onset of terminal renal failure were hypertension (72 per cent), pain (36 per cent) and urinary tract infection (32 per cent). Less common complications included haematuria, splenomegaly, gastro-intestinal disturbances and disorders of calcium metabolism. The polycystic kidney patient who is considered for renal transplantation poses questions of the desirability and timing of bilateral nephrectomy, vagotomy and splenectomy. Eight patients died without receiving a transplant, five of them from uraemia. Thirty-one patients received 36 kidney transplants and 46 per cent of these were functioning one year after transplantation. Thirteen patients who had received transplants died. Analysis of the causes of death suggests that in nearly half, major contributing factors might have been anticipated and we therefore feel that regular surveillance from the time of diagnosis is essential for patients with polycystic kidney disease.