Objective: Leiomyosarcoma (LMS), a sarcoma with smooth muscle differentiation rarely affects the oral cavity due to the paucity of smooth muscle tissues in the region. Because of the intricacy of the anatomy of the oral cavity, tumors affecting this region usually require complex surgeries. We are thus presenting this case, due to the rarity of the disease and its successful treatment by a multidisciplinary team.

Case Presentation: A 30-year-old female who was previously diagnosed with a benign smooth muscle neoplasm, had a partial maxillectomy in another institution, came to us for tumor recurrence with a histopathologic report of a smooth muscle tumor of uncertain malignant potential (SmTUMP). Facial and neck CT scan, as well as facial MRI revealed a resectable disease with unremarkable metastatic workup.

Results: The clinical aggressiveness of the disease prompted the multidisciplinary team to proceed with infrastructure maxillectomy with the defect covered by a surgical obturator. Final histopathologic report revealed that the tumor is LMS with good margins. Thereafter, the patient underwent adjuvant radiation therapy. At 1-year post-surgery, the patient had minimal speech deficit with good deglutition function and no recurrence.

Conclusions: LMS is exceedingly rare in the head and neck region. It is also difficult to diagnose. However, it should be considered a differential diagnosis when dealing with smooth muscle tumors. Successful treatment of this disease entails high index of suspicion and involvement of a multidisciplinary team.