Adult intussusception is a rare entity which is distinct from paediatric cases in incidence, aetiology, and management. It represents 5% of all intussusceptions and is the cause of 1% of all intestinal obstructions, 0,08% of all abdominal surgeries and 0,003-0,02% of all hospital admissions. Ileocolic intussusception in adults is a unique variant in which nearly 100% of cases have a malignant lead point. In our report, we described a case of a patient with ileocecal intussusception caused by a rare type of the gastrointestinal tumor. The female patient was admitted to hospital for occasional pain in the lower right quadrant of the abdomen followed by abdominal discomfort and appearance of blood in the stool. The result of CT scan of the abdomen and pelvis showed a tumor mass and intussusception at the ileocecal junction, which was confirmed peroperatively. Open right hemicolectomy was performed adhering to oncological principles. The final pathologic diagnosis indicated the gastrointestinal stromal tumor of the ileocecal valve. The diagnosis of intussusception in adults is delicate, and timely surgical treatment can be vital. Patients with the palpable abdominal mass, digestive tract obstruction, gastrointestinal bleeding, or lead point computed tomography must undergo a surgical examination. Given a high risk of malignancy, primary surgical resection using oncologic principles presents the best option for the treatment of ileocecal intussusception in adults.