Two brothers with familial carcinoid tumors of the rectum are presented. A few cases documenting the occurrence of carcinoid tumors in first-degree relatives in the absence of the multiple endocrine neoplasia (MEN) syndromes have been reported in the literature. Consistent with these previous reports, in this case both patients had gastrointestinal carcinoid tumors that are located in identical anatomic locations. The current literature on carcinoid tumors outside the setting of any known genetic syndrome is reviewed. Clinical relevance and screening recommendations are discussed.