A ganglioneuroma is a benign tumor composed of ganglion cells, nerve fibers, and supporting cells that originates from the neural crest. An occurrence of a ganglioneuroma is mainly along the pathway of the sympathetic chain. However, it is rare to find the lesion in the gastrointestinal tract. A ganglioneuroma of the gastrointestinal tract appears to involve predominantly the colon and rectum, but rarely involves the upper gastrointestinal tract. An intestinal ganglioneuroma is divided into three subgroups: a polypoid ganglioneuroma, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis. A polypoid ganglioneuroma is not associated with systemic disease. However, ganglioneuromatous polyposis and diffuse ganglioneuromatosis are highly associated with systemic diseases. A 33-year-old woman who had no specific family history visited our institution with a complaint of abdominal discomfort. A single polyp was found incidentally in the descending colon during a colonoscopy and it was treated by snare polypectomy. The patient had no associated systemic disease and histology of the polyp revealed a ganglioneuroma. We report a patient with a polypoid ganglioneuroma of the colon.