Patients with Lynch syndrome and rectal cancer present a unique clinical challenge. Management of the primary rectal cancer and prophylactic removal of the colon should be considered. In patients requiring a mesorectal excision, a combined prophylactic colon removal can be considered. Although surveillance of the colon with frequent colonoscopies is an alternative, concerns of metachronous colon cancer development support prophylactic removal of the colon as an alternative. Since data are not available to confirm superiority of either approach, the final decision is greatly dependent upon a patient’s wishes and preferences. Patients interested in pursuing simultaneous prophylactic colon removal can be offered total proctocolectomy with either ileal pouch anal-anastomosis as a sphincter-preserving alternative or a total proctocolectomy with end ileostomy.