Ganglioneuroma of the gastrointestinal tract is an extremely rare neuroectodermal tumor that can be located in the colon. In clinical practice, it is usually associated with a systemic disease. Herein, we report a case presented with intermittent rectal bleeding and finally diagnosed with ganglioneuromatous polyposis and multiple adenomatous polyps in the colon. We also discuss the patients reported in the pertinent literature.