Although most cases of intussusception during childhood are caused by viral enlargement of the Peyer's patches, the remainder of the cases may be due to congenital anomalies, such as heterotopic gastric mucosa (HGM). This report presents a case of HGM in a 4-year-old boy. The patient presented with acute abdominal signs with a right lower quadrant mass. His medical history indicated three episodes of recurrent intussusceptions. The abdominal ultrasound examination detected an intussusception. A laparotomy revealed an ileocolic intussusception and reduction was accomplished. During exploration, an intraluminal mass in the ileum, which was the lead point, was discovered by palpation. An enterotomy showed a 2-cm-diameter crater-shaped mucosal thickening. An ileal resection and anastomosis was performed. The histopathological examination revealed HGM. Heterotopic gastric mucosa is supposed to be of vitellointestinal tract origin and may cause intussusception as being a lead point in the ileum. Despite its rarity, HGM should be considered in cases of recurrent intussusceptions and diagnostic studies should be performed. A laparotomy is required for the diagnosis and treatment in complicated cases. Surgical management should include reduction of the intussusception and careful manual examination of the ileum which may expose such pathology. An intestinal resection and anastomosis is sufficient to prevent complications.