Familial adenomatous poliposis is autosomal, predominantly inherited, premalignant disease, which is caused by a mutation in adenomatous poliposis coli gene in chromosome 5q21. Intestinal transplantation is an evolving procedure and has become a lifesaving procedure for pediatrics and adults with intestinal failure who cannot be managed by conventional therapies. Long-term outcome and cost effectiveness continue to improve and will be comparable to those of total parenteral nutrition. The short gut syndrome is the most common indication. Pancreas transplantation, as a solid organ, vascularized graft, has im proved the quality of life for diabetic patients by establishing an insulin-independent, constant normo glycemic state and is a well-established treatment for patients with insulin-dependent diabetes. The bene ficial effect is the maintenance of normoglycemia and possibly the reversal of diabetic complications, such as vasculopathy, neuropathy, and nephropathy. We report a case of pancreas-intestinal transplantation in a familial adenomatous polyposis patient along with a review of the literature.