Background: Leiomyosarcoma of the uterus is a rare tumor; hence there is lack of evidence on the tumor behavior in terms of prognostic factor for recurrence and its metastatic pattern. Practitioners usually find difficulty in choosing the best management options in treating rare diseases. Case Report: A case of uterine leiomyosarcoma underwent staging laparoscopy and adjuvant chemotherapy. First recurrence was discovered as a single lung nodule after two years follow up, so the patient underwent a metastatectomy. Second recurrence was isolated right biceps muscle metastasis which was removed and followed by chemo-radiotherapy. Patient died one year following the second recurrence. Conclusion: There are no specific pathological findings or independent factors which can predict the metastatic pattern or the site of recurrence. Surgical resection appears to be the best option in management of leiomysarcoma metastasis if feasible. Evidence supports the roll of chemotherapy in non-operable recurrence.