Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease in children. The aim of this study was to determine the characteristics and outcomes of children with PSC who were listed for liver transplantation (LT). Children who underwent transplantation for PSC according to the Studies of Pediatric Liver Transplantation (SPLIT) registry were compared to agematched children with chronic liver disease who underwent transplantation for other indications. Seventy-nine patients (2.6% of the SPLIT cohort) required LT for PSC. The mean duration of the post-LT follow-up was 36.6 6 32.7 months. Ulcerative colitis and Crohn's disease were diagnosed before LT in 46.0% and 3.3% of the patients, respectively, and inflammatory bowel disease (IBD) was diagnosed after LT in another 9.8%. The mean age at LT was 12.6 6 3.9 years, and the mean waiting time was 10.2 6 12.9 months. The mean z scores for height and weight at LT were significantly lower for the PSC group versus the non-PSC group. For the PSC group, the 1-and 5-year patient survival rates were 98.7% and 86.6%, respectively, and the 1-and 5-year graft survival rates were 93.0% and 76.1%, respectively. Intrahepatic biliary strictures in the first 6 months post-LT and cholangitis in the first 30 days post-LT were more common in the PSC group versus the non-PSC group (3.8% versus 0.8% for intrahepatic biliary strictures, P ΒΌ 0.03, and 5.1% versus 1.1% for cholangitis, P ΒΌ 0.01). Recurrent PSC was diagnosed in 9.8% of the patients at a mean of 18.7 6 13.8 months after LT. IBD was associated with an increased risk of death (log-rank P ΒΌ 0.01) and recurrent PSC (P ΒΌ 0.02). Five years post-LT, the mean aspartate aminotransferase level was 60 6 45 IU/L, and the mean gamma-glutamyltransferase level was 209 6 302 IU/L; both levels were significantly higher than the levels for non-PSC patients. In conclusion, children with PSC had patient and graft survival rates equaling those of age-matched children who underwent transplantation for other indications. IBD was associated with worse outcomes. Recurrence was observed in 9.8%, and the PSC patients had higher mean liver enzyme levels 5 years post-LT.