: Neuroendocrine neoplasms are detected very rarely in pregnant women. The following is a case report of carcinoid tumor of the appendix diagnosed in 28 year-old woman at 25th week of gestation. The woman delivered spontaneously a healthy baby at the 38th week of gestation. She did not require adjuvant therapy with somatostatin analogues. The patient remained in remission. There are not established standards of care due to the very rare incidence of carcinoid tumors in pregnancy. A review of the literature related to management and prognosis in such cases was done.