Introduction and Importance:Intussusception is a rare surgical condition in adults less commonly confined to small bowel. Adult intussusception is an indication of surgical resection due to possibility of ischemia and malignant pathological cause like gastrointestinal stromal tumor (GIST) as in this case.Case presentation:A 32-year-old male presented with abdominal pain and vomiting for 3 days. Vitals parameters and abdominal examinations were normal. Abdominal ultrasonography revealed target sign suggesting ileoileal intussusception in right lower quadrant. Abdominal contrast-enhanced computed tomography of abdomen showed features suggestive of ileoileal intussusception. Diagnostic laparoscopy was done which was later turned to laparotomy for segmental resection and anastomosis of ileum for ileoileal intussusception. Polypoidal growth noted in the resected section of ileum was found to be GIST (CD117 and DOG-1 positive) which was considered to be the lead point. Patient recovered well during postoperative period and later referred to oncology clinic for chemotherapy.Clinical discussion:Intussusception and subsequent obstruction are very uncommon presentation in a patient with GIST because of their tendency to grow in an extraluminal fashion. As intussusception is rare in adult, high level of suspicion and proper imaging technique plays important role in diagnosing the condition.Conclusion:Ileoileal intussusceptions due to GIST are a rare clinical entity in adult intussusceptions and generally have a vague variable clinical presentation thus requiring high index of clinical judgement and suspicion with judicious use of imaging.