Pleomorphic sarcoma of the tongue base is an extremely rare pathology finding. Our review of current databases returned fewer than 10 articles available free full text on this subject. We review the current state of art management guidelines for this type of tumor. Our case presented surprisingly a favorable evolution despite the huge dimensions, the tumor type, and associated pathology. The patient received radiation therapy and oncological treatment followed by revision surgery consisting of partial glossectomy for the residual tumor. Histological examination of the operatory specimen showed a residual tumor of pleomorphic sarcoma type, with clear margins. The evolution was without relapse after 18 months.