Primary tumors of the small bowel are rare, and their clinical presentation is nonspecific. Consequently, preoperative diagnosis remains the exception rather than the rule. Although the small intestine makes up 75% of the length and 90% of the surface area of the gastrointestinal tract, small bowel tumors make up approximately 3% of all malignant gastrointestinal neoplasms. Primary neoplasms of the small intestine are notorious for their insidious presentation and vague symptoms, including abdominal pain, anorexia, and weight loss. These nonspecific symptoms, coupled with the lack of physical findings, often cause a significant delay in reaching a diagnosis. Because the small bowel has been a relatively inaccessible area to standard endoscopic techniques, contrast radiography has been regarded historically as the gold standard diagnostic modality. The management of primary malignant small bowel tumors is invariably surgical. However, adjuvant chemotherapy and radiotherapy may be warranted, depending on the type of tumor.