Encapsulating peritoneal sclerosis (EPS) is a severe complication of peritoneal dialysis (PD) and may present after kidney transplantation, a condition known as posttransplantation EPS. The prevalence and impact of posttransplantation EPS on survival after kidney transplantation is unknown. From January 1, 1996 until July 1, 2007, 1241 PD patients were transplanted. Thirty-eight cases of posttransplantation EPS (3%) were identified from the Dutch multicenter EPS study. In EPS patients the mean pretransplant dialysis duration was longer than in the controls (71.4 ± 37.5 months vs. 34.7 ± 25.5, p < 0.0001). The majority of EPS cases were observed within the first 2 years after transplantation, but some cases appeared many years after transplantation. Two hundred and one (16.2%) patients died after transplantation, of which 17 were EPS patients. After infection (23.9%), cardiovascular disease (21.9%) and malignancy (10.9%), EPS (8.5%) was the fourth known cause of death after transplantation. Kaplan-Meier analysis showed a significant decreased survival for transplanted patients with posttransplantation EPS compared to transplanted patients without EPS. In conclusion, posttransplantation EPS is rare but carries a high mortality. A prolonged clinical vigilance and a high index of suspicion for the diagnosis are warranted, specifically in PD patients with a relatively long cumulative pretransplant duration of PD.