It is not clear whether primary sclerosing cholangitis recurs after liver transplantation and can be distinguished from chronic rejection (CR).The purpose of this study was to determine if recurrent PSC and CR are different entities or expressions of the same disease process. One hundred consecutive patients with PSC receiving 118 grafts were retrospectively reviewed and grouped into those with cholangiographic and histologic features of PSC (Gp A, n=18), those with CR (Gp B, n=15) and those with neither diagnosis (Gp C, n=82). Significantly younger patients comprised Gps A and B but patients in Gp A had higher incidence of CMV hepatitis. 5 year graft survival was 65% for Gp A, 33% for Gp B and 76% for Gp C. 5 year patient survival was 76%, 67% and 89% respectively for Gps A, B and C. Re-transplantation rates were higher in Gp A (28%) and Gp B (47%) compared to 9% in Gp C. Patients in Gp A and B shared many histologic features. These authors conclude that although the histologic similarities suggest that CR and PSC are part of a disease spectrum, the lower graft survival and higher re-transplantation rate in CR renders them distinct entities requiring different treatment strategies.