Abstract A patient with leiomyosarcoma of the rectum is presented with a brief review of 7 other patients seen in the Birmingham region from 1950 to 1967. A short discussion is given concerning the difficulties of diagnosis and treatment. We would concur with Diamante and Bacon's view (1967) that abdominoperineal excision is the treatment of choice, but that for small border-line tumours local excision may be adequate if intensive follow-up is used. Radical excision is practised at the first sing of possible local recurrence. The Birmingham cases seem unusual because of the predominance of distant metastases when compared with the local spread more commonly reported by other workers.