Primary neoplasms of the retrorectal (presacral) space are very rare. These lesions may be congenital or acquired, benign or malignant. They often arise with subtle clinical symptoms, or they may be found incidentally during evaluation for other conditions. Preoperative imaging can provide useful information for operative planning; however, it does not eliminate the need for surgery. Approaches for resection include posterior only and combined abdominoperineal, depending on the characteristics of the lesion. Successful treatment of these enigmatic lesions requires extensive knowledge of pelvic anatomy and expertise in pelvic surgery.
Keywords: Retrorectal tumors; chordoma; presacral space; teratoma.