Abstract:Gastrointestinal stromal tumor (GIST) is a mesenchymal tissue-derived tumor that can occur in the whole digestive tract, most commonly in the stomach (60% ~ 70%), followed by the small intestine (20% ~ 30%), and only 5% in the rectum. Surgical treatment has always been the preferred treatment for rectal GIST. Traditionally, low-position anterior resection and abdominal perineal resection have been used, and the scope of resection is wide, which may cause a certain impact on the quality of patients’ life. With the deepening of the research, it has been reported that GIST rarely metastasizes to lymph node, which makes it possible for the rectal GIST to undergo local resection through the anus, the transsacral and the transvaginal approach. This type of surgical procedure has less trauma and does not cause significant damage to anatomical structures and organ function. In recent years, as molecular targeted drugs have been continuously developed and have been on the market, the tumor volume has been reduced before surgery. Therefore, more and more rectal GIST can achieve local R0 resection. However, due to the low incidence of rectal GIST, the treatment strategy for rectal GIST needs further research and discussion.