Introduction: Malignant tumors of larynx, mostly seen in males, are usually observed during the fifth and sixth decades of life. These tumors are usually associated with tobacco and alcohol usage. The symptoms of these tumors are hoarseness, dysphagia and a chronic sore throat. A rare form of laryngeal malignancy is adenoid cystic carcinoma as a malignant minor salivary gland tumor. Case Report: In this article, we report a rare case of laryngeal adenoid cystic carcinoma(ACC) in subglottic area in a 22-year-old female without any risk factors. The patient was referred suffering from pain, hoarseness and exertional dyspnea. After performing a biopsy on the subglottic mass and confirming the pathologic diagnosis of adenoid cystic carcinoma, the patient underwent total laryngectomy. During a period of 2.5-year follow-up, no sign of recurrence or distant metastasis was reported. Conclusion: The presence of upper airway symptoms needs to be evaluated. If there is no improvement in the symptoms using medical treatment, the patient should be evaluated by imaging techniques or direct or indirect diagnostic laryngoscopy. In order to prevent late diagnosis and disease progression, there should be a suspicion of malignant lesions in laryngeal tumors even at young ages.
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