Rectal gastrointestinal stromal tumors (GISTs) are rare types of tumors, but the incidence is increasing, and we now know more about the pathogenesis and management of rectal GIST. The main goal is to resect the tumor with negative microscopic margins. With the development of neoadjuvant Imatinib therapy, preoperative reduction in tumor size has become possible, thus introducing the chance for anus-preserving surgery, with better quality of life. We present a case of a 55-year-old female who presented to the emergency department with complaints of bleeding per rectum, abdominal pain, and pain on defecation. A 6-cm mass was detected during the rectal examination and a biopsy confirmed the diagnosis of GIST. The patient was given neoadjuvant imatinib chemotherapy for four months followed by trans-anal resection of the mass. The procedure was done successfully, and she received further adjuvant imatinib for a course of three years. Follow-up by magnetic resonance imaging and a colonoscopy after two years showed no recurrence. The patient is living healthily and doing well.
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