Some transplantation centers still suggest nephrectomy in patients with autosomal dominant polycystic kidney disease (ADPKD) before kidney transplantation at least in selected cases. We wanted to learn whether prior nephrectomy is beneficial. The outcome of kidney transplantation in 47 consecutive ADPKD patients without prior nephrectomy was compared with that in matched controls with respect to complications of ADPKD. Although ADPKD patients were older than controls (mean, 50.1 vs. 40.3 years), there was no statistically significant difference in 1- and 5-year allograft survival between ADPKD patients and controls: 76.6 and 68.0%, respectively, in ADPKD patients, and 83.9 and 56.3% in controls. After a mean follow-up of 66.5 months 3 patients with ADPKD had cyst infections and were managed with antibiotics. Two patients had episodes of hematuria; neither required invasive therapy. There was no renal malignancy and clinical sign of urolithiasis in any patient. No posttransplantation nephrectomy was required. With only few indications remaining, there is no rationale for routine pretransplantation nephrectomy in patients with ADPKD.