Intestinal GNs are hamartomatous polyps characterized by hyperplasia of ganglion cells, nerve fibers, and supporting cells of the enteric nervous system [
2]. Shekitka and Sobin [
3] categorized GNs of the intestinal tract into three groups: polypoid GN, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis. Polypoid GNs can be solitary or few in number. They are often small and may be sessile or pedunculated. They are endoscopically indistinguishable from hyperplastic or adenomatous polyps. Occasionally, they occur in patients with Cowden’s disease, tuberous sclerosis, polyposis coli, or juvenile polyposis [
4]. More than 20 sessile or pedunculated polyps develop in patients with ganglioneuromatous polyposis. These polyps may be an isolated finding but more commonly are identified as a component of one of the following syndromes: MEN IIB syndrome, NF, Cowden’s disease, or Ruvalcaba-Myhre-Smith syndrome. The presence of multiple cutaneous lipomas or skin tags or both has also been reported [
2,
3]. Diffuse ganglioneuromatosis disseminates to the entire colon but does not involve the ileum. Lesions can reach 1 to 17cm in diameter and may comprise multiple colonic polyps and nodular intramural or transmural proliferation of neural elements that involve the enteric plexuses. These lesions are poorly demarcated and can distort the architecture of the surrounding tissue [
5]. Although they may be an isolated finding, they are often observed as a component of MEN IIB7 or NF 1 [
6]. Hegstrom and Kircher [
7] detailed an autopsy case of diffuse gastrointestinal ganglioneuromatosis-lipomatosis. Treatment of GNs depends on the clinical history; polypectomy is curative for polypoid subgroups, but colectomy may be necessary for polyposis or diffuse forms [
8]. In this report, a solitary polypoid GN was incidentally detected during endoscopy. In most cases, a solitary polypoid GN is associated with no characteristic symptoms. However, depending on its size and anatomical location, it may cause abdominal pain, constipation, obstruction, or bleeding [
4,
9,
10]. Furthermore, GN has no typical endoscopic appearance. In this case, it was confined to the mucosa and was overlaid with normal colonic mucosa. Microscopic examination and immunohistochemistry enabled the definitive diagnosis. Stains for S100 protein and neuron-specific enolase confirm the presence of spindle cells and ganglion cells, respectively. There are no specific treatment recommendations for GNs, but they may be treated according to their size, location, and complications such as bleeding or obstruction or both. In this case, polypectomy was performed with biopsy forceps, and the six-month follow-up showed no signs of recurrence. Because of the absence of associated pathologies or symptoms, clinical management comprised a follow-up colonoscopy 18 months later. Some authors have reported the coexistence of colon cancer with GN polyposis in patients with diffuse ganglioneuromatosis [
11‐
13]. However, this association is controversial and, to some investigators, unacceptable [
14]. In a recent study of colon adenocarcinoma associated with diffuse ganglioneuromatosis, a glial cell line-derived neurotrophic factor (GDNF) and its receptor components were identified. GDNF family receptor alpha 1 (GFR-alpha 1) and the receptor tyrosine kinase (RET) are involved in the pathophysiology of both the enteric nervous system and adenocarcinoma cells, suggesting their involvement in the pathology of polypoid GN [
15]. The association between colon cancer and polypoid GN has not been studied before. The variant discussed in the present report may be cured by endoscopic resection [
9].