Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 7 1 2022 06 14 25 28 Nikhil Talwar Department of Surgery, Lady Harding Medical College, New Delhi, India Neeru Malik Department of Obstetrics and Gynaecology, Dr Baba Saheb Ambedkar Medical College, Delhi, India. Manoj Andley Department of Surgery, Lady Harding Medical College, New Delhi, India. Parul Talwar Department of Obstetrics and Gynaecology, Bhagwan Mahavir Hospital, Delhi, India. 2021 03 14 2021 12 01 Presacral tumors are a rare group of tumors located posterior to the rectum in the retrorectal or presacral space. Due to the lack of specific symptoms and difficult anatomic localization, they present a diagnostic and management challenge for the surgeons. We present a case of retrorectal schwannoma in a young female who was initially misdiagnosed as a uterine fibroid on ultrasound and taken up for myomectomy. The tumor was later successfully excised by a combined abdominosacral approach. The clinical features and management of presacral tumors are discussed in this article. https://crcp.tums.ac.ir/index.php/crcp/article/view/495