Conclusion
We reported a case of metastatic euLMS with uncommon metastatic sites, pancreas and gut. The metastatic tumors surgically resected then adjuvant gemcitabine plus docetaxel chemotherapy was applied for this patient, given the rarity of such patients and lack of guidelines for oncological therapy of such cases, we suggest that multimodal therapy, by a multidisciplinary team, is essential to achieve an optimal outcome.