The authors report a new case of leiomyosarcoma of the oesophagus, and a review of the literature since 1960 (during this period almost every case has been resected). Four macroscopic aspects are observed: polypoid, mediastinal, intra-mural and infiltrating. The accurate diagnosis is difficult and was established before the operation only in half cases. It is based on barium swallow examination and esophagoscopy, that reveal different appearances depending on the anatomical type, and on the biopsy. The interpretation of the biopsy may be difficult because of the superficial nature of the sampling, frequently taking only mucosa or necrotic tissue. It is necessary to remove the necrotic tissue to discover the tumoral tissue, or to perform a wide diathermic snare biopsy. The diagnosis of malignancy is mainly based on the mitosis count, and interpretation of the peroperative pathologic examination is difficult. The surgeon may have to decide on an esophageal resection, according to the gross appearance. Esophagectomy is the most logical treatment; adjuvant radiation therapy could have some efficiency.