We reviewed 45 patients who underwent surgery for primary jejunoileal tumors over a 15-year period. There were 16 benign and 29 malignant tumors, which included 13 lymphomas, 7 adenocarcinomas, 7 carcinoid tumors, and 2 leiomyosarcomas. Eighteen patients, 13 of whom had benign tumors, presented with intestinal bleeding and 5 tumors were found incidentally at laparotomy. Benign lesions were more frequently sited in the jejunum while malignant lesions were more common in the ileum (p less than 0.001). Lesions presenting with hemorrhage were more likely to be benign than malignant (p less than 0.001) and were more commonly sited in the jejunum than in the ileum (p less than 0.05). Visceral perforation (31%), intestinal obstruction (21%), and an abdominal mass (17%) were other presenting features in patients with malignant tumors. In spite of a wide variety of investigations, the correct diagnosis was reached preoperatively in only 31% of patients. Surgical management included either limited bowel resection or segmental resection with regional lymphadenectomy. Operative mortality was 13% and morbidity was 36%. Actuarial 5-year survival for all malignant tumors was 24%, being 64% at 5 years for carcinoid tumors, 20% at 30 months for adenocarcinomas, and 10% at 42 months for lymphomas. These results reemphasize the need for a high index of suspicion and early laparotomy in patients with obscure intestinal symptoms if the prognosis of small bowel tumors is to improve.