We herein report a case of a 14-year-old male with mucosa-associated lymphoid tissue (MALT) lymphoma of the ileum as the cause of an intestinal intussusception. The patient was referred to the hospital with abdominal pain and dyspeptic complaints and was operated on with a prediagnosis of acute abdomen. Abdominal exploration revealed an ileoileal intussusception induced by a 2-cm tumor. A segmental ileal resection was done at the operation. The histopathologic examination of the specimen revealed atypical lymphocytes starting from the mucosae infiltrating the muscular layer and the serosae. Based on these findings, the tumor was diagnosed as a high-grade MALT lymphoma. The patient recovered uneventfully after the surgery, and chemotherapy was started.