Tumors of the small intestine offer a unique challenge. As a result of their infrequent occurrence, they invariably present difficult problems in diagnosis and management. Although the prognosis for benign lesions is excellent, malignant small bowel tumors are perhaps the most devastating GI malignancies; at the time of diagnosis, only approximately 50% of these lesions are completely resectable for cure. Symptoms are often absent until the tumor has progressed to produce a complication. Even then, the presentation is often vague and nonspecific, intermittent pain, obstruction, and chronic anemia. The cornerstone of diagnosis is the contrast radiograph. However, in practice only about 50% of these lesions are diagnosed radiographically before surgery. This situation is further complicated by the variety of small bowel tumors, each with different symptoms and manifestations. Surgical excision is the treatment of choice for almost every small intestinal neoplasm. For most benign lesions simple excision is adequate. In contrast, for malignancies, segmental resection including as much adjacent mesentery as is reasonable, is required. In the duodenum, these tumors may necessitate pancreaticoduodenectomy; in the ileum, right colectomy may be required. In the case of advanced disease, palliative resection to relieve bleeding or obstruction may be indicated. The challenge of the future will be to reduce the morbidity and mortality of small bowel neoplasms not only by earlier recognition, diagnosis, and therapy but also through the development of alternative or adjunctive therapy for patients in whom surgical cure is not possible. This will require not only a high index of suspicion when confronted with patients with vague abdominal complaints but also an aggressive approach to diagnosis in the face of normal initial studies. In addition, multi-institutional trails of chemotherapy and radiation therapy of these tumors are needed.