Although various forms of polycystic kidney disease (PKD) account for up to 10% of patients requiring renal replacement therapy and severe complications may arise from these kidneys, no clear indications for pretransplant nephrectomy have been defined so far. A total of 104 renal transplants in three pediatric and 96 adult patients suffering from PKD were analysed retrospectively with regard to patient and graft survival in relation to pretransplant or posttransplant nephrectomy and no nephrectomy. Of these 99 patients, 25 had had either unilateral (19) or bilateral (6) nephrectomy sometime before transplantation and 10 patients between 3 and 81 months after transplantation. All patients received Cyclosporine-based immunosuppression. One-year patient and graft survivals for recipients of a first cadaveric renal graft (n = 91) were 94% and 92%, for recipients of second or third graft (n = 13) 89% and 78%. One- and five-year patient survival rates for patients with or without pretransplant nephrectomy were 100% and 100% vs 92% and 84%, respectively. One- and five-year graft survival rates were 100% and 93% for pretransplant nephrectomy patients vs 89% and 74% for the non-nephrectomy group (p < 0.05). Patients not undergoing nephrectomy sometime after transplantation had the same patient but better five-year graft survival when compared to the posttransplant nephrectomy group (89% vs 52%). In patients with early posttransplant urinary tract infection, which is considered in this analysis as a cyst-related complication, graft survival at one year was 77% but 97% in patients without this complication. From these data it is recommended that polycystic kidneys should be removed before transplantation if cyst-related complications occur repeatedly. Posttransplant nephrectomy can be performed with no mortality and should be carried out whenever clinically indicated.