Fifty-five patients undergoing orthotopic liver transplantation (OLT) for primary sclerosing cholangitis (PSC) were reviewed retrospectively. After OLT there were significantly more episodes of acute rejection in 31 patients with a previous history of inflammatory bowel disease (IBD) than in 24 without such a history. Patients who had severe acute rejection were significantly younger at diagnosis of IBD. Five patients with histological features suggestive of recurrent PSC after OLT were significantly younger at the time of transplantation than those who did not develop recurrence. Patients with poor control of IBD after OLT had a significantly lower age at diagnosis of bowel inflammation. The presence of IBD did not affect the outcome after OLT, but was associated with a higher rate of acute rejection. Younger patients had a greater risk of developing severe rejection, recurrent PSC and poor control of IBD after liver transplantation.