Autosomal dominant polycystic kidney disease (ADPKD) is a common cause of end-stage renal disease and a common indication for renal transplantation. Recipients of renal grafts with ADPKD show some differences in graft outcome and complications. In this study, we evaluated the demographics, outcomes and complications of renal transplantation in patients with ADPKD. In a retrospective case-control design, 51 patients with ADPKD were recognized among all 1200 renal transplant patients. For each case, a matched control based on sex, age (±5 years) and kidney donor, was selected. All demographic and operation data was gathered using patients' records and PNOT software. There were 34 males (66.7%) and 17 females (33.3%) with ADPKD. Mean age at transplantation was 42.6±14.3 years and source of organ transplantation was predominantly live unrelated (72.5%). Forty patients (78.4%) presented with extrarenal manifestations of ADPKD, the most common of which were: cardiac valvular disease (24 cases, 47.1%), and liver cysts (10 cases, 19.6%). Rejection occurred in 12 patients in case group (23.5%) comparing to 9 patients (17.6%) in the control group (p>0.05). Twentynine cases (56.9%) were free of any complications. Common complications included infections (15.7% in cases vs. 19.6% in controls), and cerebrovascular accidents (13.7% in cases vs. 16.6% in controls). Extrarenal manifestations were not different from other studies. Patient outcome was slightly better in the ADPKD population than the control group in short- and long-term follow-up; however, it was not statistically significant. In contrast to other studies, no complication was found to occur more frequently in ADPKD patients.