A case of multiple carcinoid tumors of the rectum with numerous proliferations of extraglandular endocrine cells is reported. The patient was 52-year-old man with five polypoid lesions in the rectum. The resected rectum contained five macroscopic carcinoid tumors, 36 microcarcinoids, and innumerous extraglandular endocrine cell proliferations. Endocrine cell microproliferations, in their early stage consisting of one to 15 micronests, were mainly located within the bundles of muscularis mucosae, having no contact with mucosal glandular structures. All of the immunohistochemically examined proliferations of extraglandular endocrine cells contained S-100 protein-positive dendritic cells, and some endocrine cells coexisted with submucosal ganglion cells. In contrast, there was no increase in intraglandular endocrine cells. The origin of rectal carcinoid tumor may be the extraglandular endocrine cells, a distinct compartment of mucosal endocrine cells of the rectum.