Leiomyosarcoma of the rectum is rare. The standard treatment is abdominoperineal resection and, in the past, radiation therapy has been employed in the palliative setting only. The authors report two additional cases of this rare tumor and its treatment using sphincter-preserving surgery and radiation therapy. In selected patients, conservative surgery followed by radiation therapy (brachytherapy with or without external beam radiation) may be an alternative to radical surgery with the goals of local control of the disease and rectal sphincter preservation. However, more experience and longer follow-up are needed before this approach could be recommended routinely.