A 33-year-old woman sought medical care at our institution for macroglossia. She had been treated before with tracheostomy and gastrostomy due to an adenoid cystic carcinoma (ACC) of the mobile tongue, in clinical stage IVC. She was subjected to total glossectomy with larynx preservation, modified radical dissection of the right neck and of the left supraomohyoid muscle, as well as reconstruction using a free flap from the rectus abdominus. The histological report described a 15x11 cm solid type ACC of the mobile tongue (grade III), with infiltration to adjacent soft tissues, vascular and lymphatic vessels, as well as two metastatic lymph nodes in the right neck dissection. One month after surgery, bone and bilateral pulmonary tumor activity became evident. In advanced-stage tumors, treatment must be individualized, and when accompanied by severe deterioration of the quality of life, surgery is recommended independently from the extension, provided that a good reconstruction is accomplished.