Starting from personal observation of a case of congenital stenosis of the oesophagus, the Authors proceed to a review of the scanty literature on the subject. After a discussion of the most outstanding aspects of differential diagnosis, the frequent necessity of histological verification, especially in adolescence and adult age, in order to obtain a correct understanding of the nature of the case, is stressed. On the basis of an essentially favourable prognosis, the Authors maintain that, where medical and dilating treatment is insufficient, surgery is indicated and evidence the straight-forward nature of the process of exeresis in stenosis of valvular type.