The authors describe the anatomo-clinical characteristics of a case of leiomyosarcoma of the oesophagus which came under their observation, emphasizing its rarity: 39 cases in the literature examined. They then discuss the numerous problems that this neoplasia involves, especially diagnostic differentiation from other sarcomas and the pseudo-sarcomatous reaction of the stroma during the course of carcinomas. Lastly they divide leiomyosarcoma of the oesophagus into polypoid forms and infiltrating forms, owing to the different therapeutic and prognostic implications that such distinction implies.