INTRODUCTION This study examined the clinical indications and timing for native nephrectomy (NN), together with the associated pathological findings in transplant patients with autosomal dominant polycystic kidney disease (ADPKD) at our institute over a period of 20 years. METHODS A retrospective review was performed of ADPKD patients who had undergone both kidney transplantation and NN. Patients were identified from the kidney transplant database between 1988 and 2008 at Guy's and St Thomas' Hospital and the notes reviewed. All NN specimens were re-reviewed and reported according to current guidelines. RESULTS There were 157 kidney transplants performed for ADPKD (114 cadaveric and 43 living donor). Of these, 31 required NN (28 bilateral). The timing of NN was pre-transplant in 10 cases, at the time of the transplant in 1 case and post-transplant in 20 cases. The indications for NN were urinary tract infection (n=14, 45%), pain (n=12, 39%), tumour suspicion (n=3, 10%), haematuria (n=1, 3%) and space (n=1, 3%). Mortality in this NN series was 3%, with a 65% surgical morbidity rate. The length of hospital stay post-NN was significantly longer with open compared with laparoscopic techniques (p=0.003). There were two renal cell carcinomas (RCCs) in this series. Both patients presented with macroscopic haematuria (bilateral pT1a papillary RCCs in one case and a pT3b clear cell RCC in the other case). The incidence of RCC in this series of ADPKD transplant patients was 1.3%. CONCLUSIONS We have demonstrated that the majority of ADPKD patients do not require NN, with only 20% of our series undergoing this procedure. The timing of NN is variable and dictated by indication. NN was only required to make space for transplantation in one case (combined kidney and pancreas transplant). The main indications for NN were recurrent infection and pain, where NN can provide a successful outcome. Laparoscopic NN can be performed safely in patients with ADPKD. Haematuria in such patients should not be assumed to be of benign origin and requires exclusion of urinary tract malignancy as the incidence of RCC in this population is at least as common as in the general population.