The size and depth of invasion are the most important prognostic factors. Since they grow within the intestinal wall, the symptoms are usually few or late, leading to delays in diagnosis. Most common signs are rectal bleeding, abdominal or anal pain, diarrhea, tenesmus and weight loss. The diagnostic and staging protocol of stromal tumors of the rectum includes mainly endoscopic surveillance, computed tomography, and magnetic resonance imaging. Therefore, rectum sarcoma (RS) consists one of the most biologically virulent cancers and is difficult to cure by conventional procedures. The treatment is primarily surgical, where possible, and should guarantee complete clearance of the tumor, which often requires an aggressive approach. Unfortunately, the minority of patients is eligible to undergo surgical intervention. In addition, surgical removal of RS does not necessarily indicate a patient's long-term recovery. Alternative therapies, such as radio- and chemotherapy, proved insufficient. Elucidation of its molecular basis may prove useful in developing and identifying prognostic biomarkers.