Background: End stage renal disease (ESRD) patients with autosomal dominant polycystic kidney disease (ADPKD) required native nephrectomy because of various indications. However, the appropriate timing for nephrectomy and also its effect on allograft and patient survival has not been fully investigated. Methods: We retrospectively analyzed 41 kidney transplant recipients with ADPKD in whom transplantation was done either simultaneously, after or without native nephrectomy at Seoul St. Mary's hospital between January 1987 and February 2014. We divided patients into 2 groups; simultaneous nephrectomy group (group A, n=13), after or without nephrectomy group (group B, n=28) and compared the perioperative outcome, post-transplant complications, allograft and patient survival rate. Results: The mean operative time was significantly longer in group A than group B. (6.48±1.84 vs. 5.27±0.84 hours; p=.048) The mean intraoperative blood transfusions was also significantly more needed in group A than B (3.66±3.43 vs. 0.75±0.26 units; p=.018). However, there were no differences in the incidence of acute rejection and other complications such as post-operative bleeding, infectious complication between the two groups (p>.05, for all) The graft and patient survival rate also did not differ between the two groups (p>.05, for all). Conclusions: Our study suggests that the complication rates were acceptable and there was no significant negative impact on graft and patient survival rate when native nephrectomy was done during kidney transplantation in ADPKD patients with ESRD. Therefore, if native nephrectomy is needed in ADPKD for kidney transplantation it can be done safely during transplantation.