We describe the case of a 31-year-old Japanese man with familial amyloid polyneuropathy combined with Crohn's disease, who underwent living-related orthotopic liver transplantation. Although he had a 2-year history of alternating constipation/diarrhea, his bowel symptoms exacerbated severely during the period he was in our ward for pretransplant evaluation. Small bowel barium examination and gastroduodenal endoscopy showed typical features of Crohn's disease. He was diagnosed as having active Crohn's disease, and the scheduled living-related orthotopic liver transplantation was postponed. After controlling the active disease with conservative therapy for two months, he underwent living-related orthotopic liver transplantation using a graft of the left lobe from his father. Postoperative medication consisted of azathioprine, aminosalicylates, tacrolimus and steroids. His postoperative course was uneventful, and he was discharged from hospital on the 25th postoperative day. Pre- and postoperative transplant management of patients with Crohn's disease is also discussed, because it influences the clinical course of inflammatory bowel diseases.