BACKGROUND: Carcinoid tumors are the most frequently encountered endocrine tumors of the gastrointestinal tract. They are most often found in the appendix, although they can arise in any location of the gut. Carcinoid tumors may secrete a variety of bioactive substances, which can cause the complex of symptoms associated with the carcinoid syndrome. METHODS: The authors reviewed the pathology, clinical presentation, and management of carcinoid tumors with an emphasis on the surgical management. RESULTS: The primary treatment for a carcinoid tumor located anywhere in the gut is surgical. Those who have widely metastatic disease or who are anatomically unresectable may undergo cytoreductive surgical debulking and/or hepatic arterial embolization followed by palliation of symptoms with octreotide, the long-acting somatostatin analog. CONCLUSIONS: The prognosis for patients with carcinoid tumors that are fully resected is excellent. Those with hepatic metastases and the carcinoid syndrome have a less favorable prognosis.