Carcinoid tumors are rare, slow-growing tumors found primarily in the GI tract. Carcinoid syndrome develops when vasoactive substances (particularly serotonin) released by carcinoid tumors gain access to the systemic circulation. Carcinoid heart disease develops in patients with carcinoid syndrome and is commonly associated with the development of right-sided valve dysfunction and signs of symptoms of right heart failure. Timely surgical intervention provides relief from symptoms and may improve survival. Management of these patients should be undertaken in a specialized center by a multidisciplinary team with appropriate expertise.