Carcinoid tumor arising in a Meckel's diverticulum is a rare lesion with only 52 cases described in the literature. Most of these tumors are found incidentally at autopsy or during an operation performed for an unrelated reason. The lesion can, however, in about one-third of cases, be the cause of symptoms necessitating surgical intervention; the diagnosis is nearly never apparent till the specimen has been removed. When the signs do not match the symptoms, consideration of uncommon conditions such as this may help in making a correct preoperative diagnosis. Resection of the intestinal segment containing the diverticulum along with its mesentery to the level of the superior mesenteric vessels usually results in cure; however, metastatic disease and even carcinoid syndrome may still occur. Second primary malignancies are often found in patients with carcinoid of Meckel's diverticulum.