The role of restorative proctocolectomy with ileal J-pouch anal anastomosis (IPAA) is uncertain for patients with ulcerative colitis (UC), when advanced lower rectal cancer is diagnosed. We report what to our knowledge is the first documented case of successful preoperative chemoradiotherapy followed by IPAA with partial intersphincteric resection of advanced rectal cancer associated with UC. A 59-year-old woman with a 24-year history of extensive UC was found to have advanced rectal cancer located 2 cm from the anal verge. She underwent preoperative conventional chemoradiotherapy followed by restorative proctocolectomy with total mesorectal excision. The procedure included intersphincteric resection of one quadrant and construction of an IPAA with diverting ileostomy. The postoperative course was uneventful, and the ileostomy was closed 6 months after the initial surgery. The patient was doing well with good pouch function and no evidence of recurrent disease 1 year after her initial surgery.