This case demonstrates the successful treatment of a young female patient with colitis cystica profunda causing rectal prolapse, after primary treatment with a Delorme procedure had failed. An ultra-low anterior resection with a temporary defunctioning ileostomy was carried out with good postoperative results. This case illustrates the possibility of carrying out sphincter preserving surgery rather than an abdominoperineal resection in the treatment of this condition, which may be preferable for patients.