The authors report a case of localised intestinal lymphangiectases in a segment of jejunum with the unusual presentation of an acute perforation. The anatomical findings did not provide a satisfactory aetiopathogenic explanation, although the most likely hypothesis is a past history of abdominal irradiation. The mechanism of the perforation also remains unclear and the presence of microscopic lesions in the wall of the remaining small intestine raises the problem of the long-term prognosis.
