A case of inflammatory bowel disease (IBD) complicating neuroendocrine neoplasms (NENs) was reported and 69 cases in references were reviewed to analyze the clinical features of IBD complicating NENs and to explore the connection between IBD and NENs. Thirty-two cases of Crohn disease (CD) and 37 cases of ulcerative colitis (UC) were included in the study. The occurrence rate showed no significant difference between males and females (P=0.151). NENs mostly occurred after the diagnosis of IBD. The median interval duration of NENs after CD was 4.5 years，which was significantly shorter than that of UC (17 years, P=0.002). Thirty-three cases discovered NENs occasionally with no special indications. Among those symptomatic patients, 11 of them suffered from intestinal obstruction. The location of NENs was similar to IBD, that was, ileum and appendix in CD (27 cases) while colon and rectal in UC (31 cases, P<0.001). Neuroendocrine tumors were more common in CD (26 cases) while neuroendocrine carcinomas were more common in UC (22 cases, P<0.001). There is possibility that IBD complicate with NENs with no specific clinical features. The etiology of this phenomenon is still not clear, which needs further exploration.