From 1950 through 1974, a total of 108 cases of primary intestinal leiomyosarcoma were seen at the Mayo Clinic. Most of these uncommon tumors occurred in the fifth and sixth decades of life, and more of them in men than in women (2.6:l). There were 73% in the small bowel, 25% in the large bowel, and 2% in the anus. Gastrointestinal bleeding and pain were the two most common signs at presentation, and they led to surgical exploration in all cases where they appeared. By the time surgery was performed, only 48% of the tumors could be resected with hope of cure. Within that group of cases, 50% of the patients survived 5 years, but only 35% survived 10 years, late recurrence being common. The histologic grade of the tumor affected survival strongly. Lack of recognition of the high late recurrence rate probably led to erroneous early optimism in prognosis.