Objective: Lipomatosis of ileocaecal valve is a rare cause of small bowel obstruction. Method: We describe a 41-year-old male treated for Crohnś disease and clinical and imaging presentation of chronic small bowel stenosis and obstruction. Results: A right hemicolectomy was performed, and hypertrophy of Bauhin´s valve causing intestinal obstruction was found. Microscopical examination of the specimen revealed morphological changes consistent with lipomatosis of the ileocaecal valve. The ileum and colon were macroscopicaly unremarkable and histomorphological features of Crohn´s disease were absent. Conclusion: Many patients with lipomatosis of the ileocaecal valve are asymptomatic, or the lipomatosis gives causes insignificant symptoms only or rarely obstruction may occur. Then, as in the presented case, the surgical resection with ileo-colic anastomosis is the only effective treatment.