A previously healthy 25-year-old white male presented with complaints of abdominal pain for two weeks. The pain was mainly located in the periumbilical area and was associated with nausea. There was no fever. The pain progressively increased and the patient started presenting episodes of vomiting. Abdominal radiographs and ultrasound scans revealed findings of intussusception. Laparotomy was performed and ileal intussusception was found 60 cm from the ileal-cecal valve, caused by a 5-cm tumor involving the intestinal wall of the ileum. The tumor was surgically removed and the patient's postoperative evolution was uneventful. Clinical information was obtained from the patient's records. The gross pathological examination revealed an ulcerated polypoid tumor of the ileum measuring 5 cm across the greatest diameter. The cut surface showed a bright whitish tumor mass with areas of hemorrhage. The diagnosis was based on examination of histological sections stained with hematoxylin and eosin. Microscopic examination revealed a neoplasm involving the submucosa and the muscle layer of the ileum. The tumor was composed of bland spindle cells with ovoid to elongated nuclei and indistinct cytoplasm. It was highly vascularized, with the presence of many small rounded vessels. There was a tendency for the tumor cells to be located around these vessels in whorls of striking appearance (). No clear-cut atypia or pleomorphism was seen. In some areas, the tumor seemed to infiltrate the muscle layer focally. The stroma was hyalinized in some areas and myxoid in others. The expression of epithelial membrane antigen (EMA), claudin-1, S-100 protein, CD117, CD34, cytokeratin and smooth-muscle actin and desmin was investigated by means of immunohistochemistry using a standard avidin-biotin method. Immunohistochemical analysis revealed diffuse expression of EMA () and claudin-1 by the neoplastic cells, but the cells were negative for S-100 protein, CD117, CD34, cytokeratin, smooth muscle actin and desmin.