Background and study aims: Hypertrophy of ileocaecal sphincter seems to be the basic etiological factor of Bauhin’s valve syndrome (BVS). In the rare literature all cases are treated by means of an hemicolectomy. A patient with Bauhin’s valve syndrome is described, whose pathologic characteristics were hypertrophy of ileocaecal sphincter and a circular submucosal lipoma on the caecal side of the valve. Lipomata, although uncommon, may arise throughout the whole gastrointestinal tract, mostly asymptomatic, and submucosal layer is most frequently involved than subserosal one. More than two-thirds of gut lipoma are found in the large bowel, where they represent the most common benign lesion after adenoma. Patients and methods: A 36 year old woman suffering from periodic upper abdominal pain, nausea and diarrhea, was bubmitted to an explorative surgical procedure, after imaging study of the bowel showed only an indistict mass in the caecum. Though a caecotomy a dissection of an hypertrophic and swollen portion of the ileocaecal valve was performed, saving the ileo-caecal sphincter. The residual mucosal margins were sutured, the last tract of the ileum was fixed to the caecum, completing with a caecorrhaphy and appendectomy. Results: Normal post-operative period, discharging the patient after few days. Since the operation all symptoms disappeared, and after four years there is a total well-being of the patient with complete disappearance of the former symptoms. Conclusions: If the BVS is only due to a lipomatous hypertrophy of the mucosa and submucosa of ileo-caecal valve, hemicholectomy seems to be not justified: it is an exceedingly invasive procedure for a non-malignant disease. It is important a throughout radiological diagnosis and a caecotomy direct control during an operative exploration for a correct surgical choice.