Gastrointestinal stromal tumor (GIST) is a group of tumors which is independently derived from the mesenchymal stem cells of the digestive tract, and consists of undifferentiated or multi　functional spindle or epithelioid cells. Most of the rectal stromal tumor located at the middle or lower part of the rectum, and presented with exogenous growth outside of the rectum cavity. Rectal stromal tumor accounted for 5% of GIST. Rectal stromal tumor is easily missed or misdiagnosed because of insidious onset and non　typical clinical manifestations. The diagnosis of rectal stromal tumor relies on immunohistochemical staining (CD117 and CD34) and pathological examination. Surgical resection is the first choice for the treatment of local primary rectal stromal tumor. Protection of the integrity of the tumor is important during operation, and routine lymph node dissection is not recommended. Molecular targeted drug mesylate imatinib is  adopted in the pre and postoperative adjuvant therapy. However, due to the rarity of rectal stromal tumor, the treatment strategies need further investigation.