Adenoid Cystic Carcinoma of larynx is extremely rare location. We herein describe an unusual clinical and radiological presentation of ACCL and review recent literature. We report a case of a 38-year-old woman with history of asthma, presented to our department with acute inspiratory dyspnea that required an emergency tracheotomy. Physical examination revealed a large anterior cervical mass without any lymphadenopathy suspecting thyroid origin. Cervical Computed Scan showed a tumor process between the thyroid lobe, the left edge of the subglottic area and first tracheal rings filling all the lumen, discussing either a laryngo-tracheal or thyroid origin. The patient underwent a panendoscopy under general anesthesia that confirmed a subglottic extension of the tumor and multiples biopsies showed a malignant salivary origin of the mass. After multidisciplinary discussion, the patient underwent total laryngectomy and thyroidectomy with bilateral selective neck dissections (levels II- IV). Anatomopathological examination confirmed the laryngeal location of Adenoid Cystic Carcinoma classified pT4aN0R0. Adjuvant radiation therapy was indicated. In our knowledge, only 10 cases were reported in the literature with this unusual presentation. Moreover, the case we report is in the subglottic floor without invasion of neither vocal cords nor trachea. Total laryngectomy with neck dissection remains the recommended therapeutic procedure for locally advanced ACCL. Adverse features such as close or positive margins, T3-4, intermediate or high grade neural and perineural spread, lymphatic or vascular invasion or lymph node metastases should indicate adjuvant treatment to improve the outcome. The lack of randomized multicentric study, implies the management of ACCL by skilled multidisciplinary team, to suggest adequate personalized treatment.