Rectal leiomyosarcoma is a very rare entity. Surgery is the main treatment, but the place of radiation therapy remains unclear. A 67-year-old woman was referred for a few-weeks' history of bleeding and anal pain intensified during defecation. Pelvic magnetic resonance imaging (MRI) showed a rectal lesion and biopsies revealed a leiomyosarcoma of the lower rectum. She was free of metastasis on computed tomography imaging. The patient refused radical surgery. After discussion by a multidisciplinary team, the patient received pre-operative long-course radiotherapy followed by surgery. The tumor was treated with 50Gy delivered in 25 fractions, within 5 weeks. The aim of radiotherapy was local control, allowing organ-preservation. Four weeks after radiation therapy, organ-preservation surgery could be performed. She had no adjuvant treatment. At 38-months follow-up, she had no local recurrence. However, distant recurrence (lung, liver, and bone) was detected 38 months after the resection and was managed by intra-venous doxorubicin 60mg/m ² and dacarbazine 800mg/m ² every 3 weeks. The patient was in a stable condition for nearly 8 months. The patient died 4 years and 3 months after the diagnosis.