Introduction The Soft tissue sarcomas (STS) are exceptionally rare mesenchymal derived tumors accounting for only 1% of all malignancies. Leiomyosarcoma (LMS) include 5-10% of STS cases that displays heterogeneous subtype of malignant mesenchymal tumor originated from smooth muscle tissues 1. The LMS Commonly diagnosed in the fifth and sixth decades of life, and it can be detected every anatomic site, including the uterus, retroperitoneum, extremities, and vasculature 2. The two primary categories of LMS contains uterine LMS (uLMS) and extrauterine (euLMS) types, the first is commonest subtype of uterine sarcoma, while metastatic euLMS are not well described in terms of treatment, outcomes and prognostic factors3. The treatment of this tumor is controversial. However, regardless the site of origin, surgical resection is cornerstone treatment for localized LMS 2,4. The standard surgical procedure includes a complete excision with wide negative margins that gives the best chance of cure 2. Pelvic external beam radiation therapy with or without brachytherapy are recommended for locally resected tumors, especially for advance metastatic disease, chemotherapy considered as an option2,4. In the euLMS is not clear that the choice of specific agent or sensitivity to first-line systemic therapy affects the response rate and clinical outcomes 5.The molecular heterogeneity of leiomyosarcoma is uknown also targeted therapy not available now, detection of different molecular subtypes is urgent to weigh novel therapeutic options. Two diagnostic immunohistochemical markers newly detected in formalin-fixed, paraffin-embedded tissues; are LMOD1 (Leiomodin 1) in subtype I and ARL4C (ADP-ribosylation factor-like protein 4C) in subtype II leiomyosarcoma 6,7. Acording leiomyosarcoma tissue microarray and considering clinical outcome, specified that subtype I leiomyosarcoma is associated with good outcome in extrauterine leiomyosarcoma nonetheless, subtype II is related to poor prognosis in uterine and extrauterine leiomyosarcoma 7.Due to rarity and few confirmed cases reported in the literature, this case report presents a young female patient with euLMS, invading the ovary, uterus, small intestine, sigmoid colon, rectum, omentum and pancreas, which were treated with radical surgery and adjuvant chemotherapy.