Study Design. Report of a patient with a carcinoid tumor of the coccyx. Objectives. To describe the clinical presentation, diagnosis, and treatment of a patient with a carcinoid tumor of the coccyx and to review the relevant medical literature in English. Summary of Background Data. No reports of a carcinoid tumor of the coccyx were found in the literature. Seven reports of carcinoid of the sacrum are described. Methods. Clinical history, magnetic resonance imaging studies, and light and electronic microscope micrographs are reviewed. Results. A coccygeal mass was detected during evaluation of coccygodynia in a 40-year-old woman. Four years after extended coccygectomy, there are no signs of local tumor recurrence. Conclusions. Carcinoid tumor of the coccyx is extremely rare. An extended coccygectomy may lead to a cure or at least to a prolonged disease-free interval.