BACKGROUND AND AIMS: The NMVH of the intestine is a rare lesion, typical located in the small bowel. After the first 2 cases, reported in 1982, other 12 cases have been described, ten localized in the jejunual or ileal tract, only 1 in the cecum. SUBJECTS AND METHODS: A case of NMVH of the duodenum, simulating a Crohn’s disease, is described. The lesion extended about 12 cm. and it caused intestinal stenosis. The microscopic analysis on the operative specimen highlighted the presence of a neuronal hyperplasia at mucous membranae and submucosal levels, associated to muscolaris mucosae hypertrophy and disorganisation. Expanded and ectasic veins, proliferation of nervous fibres and ganglion cells in the submucosal were also observed. RESULTS: NMVH is a non-epithelial hamartomatous, submucosally based proliferation of mature submucosal elements capable of causing small bowel obstruction. In the controversy about the nature of the NMVH, we consider it a separate entity, where the final diagnosis depend on the clinicopathologic setting, as well as on the location and number of the lesions. CONCLUSIONS: The Authors analyse the main characteristics of this rare pathology with regard to clinical, diagnostic and therapeutic aspects.