Sarcomas represent 1-2% of all uterine malignancies and leiomyosarcomas (LMSs) are the most common histological subtype, accounting for nearly 30% of all uterine sarcomas. The diagnosis is incidental following routine hysterectomy, and the disease exhibits a high rate of relapse and distant metastases. The most common location for metastasis is the lung, while the finding of bone metastasis in the oral region is exceedingly rare. Here the authors report the case of an exceedingly rare mandibular disease recurrence from a previously uterine LMS refractory to multiple treatments, which was beneficially managed by systemic trabectedin administration. The authors also discuss the features connected with the diagnosis and management of such an uncommon presentation of the metastatic disease.