The total number of patients undergoing chronic dialysis treatment at the end of 2019 in Japan was 344,640. Autosomal dominant polycystic kidney disease (ADPKD) is the fourth leading causative disease requiring dialysis treatment and 930 of ADPKD patients started dialysis treatment in 2019. Survival of ADPKD patients on renal replacement therapy is higher than that of non-ADPKD patients. Kidney transplantation in ADPKD has been also demonstrated better outcomes than those of non-ADPKD patients, but we have to be care of some critical points related to prepare and to perform kidney transplantation. In the case of a living related donor, exclusion of the presence of ADPKD in the potential donor is mandatory. Secondary, elective nephrectomy of native kidneys should be considered prior to kidney transplantation when the kidney size prevents adequate placement of the graft. Timing (pretransplant or simultaneous with transplantation) should take into account the experience of each center. Native kidney nephrectomy may also be indicated to treat complications such as bleeding, persistent infection, and intolerable pain. Furthermore, unilateral or bilateral native kidney nephrectomy is associated with a better control of hypertension in ADPKD after kidney transplantation. We analyzed our kidney transplantation activities between December 2012 and June 2021 regarding ADPKD. Among the 140 kidney transplantations, 16.2% (n=23) had an ADPKD. All cases were living-related-donor kidney transplantations and 56.5% (n=13) were pre-emptive kidney transplantations. Since we started a special outpatient clinic for ADPKD patients to treat with tolvaptan in 2014, we would say that the fact that we were able to collect a relatively large number of ADPKD patients has increased the proportion of preemptive kidney transplantations.