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Background and Clinical Significance: Adenoid cystic carcinoma (ACC) is a rare neoplasm of salivary glands, accounting for approximately 2-4% of all ACCs of head and neck malignancies. Adenoid cystic carcinoma (ACC) of the larynx is exceedingly rare, accounting for only 0.07-0.25% of all laryngeal tumors. Within the larynx, ACC may arise in various locations; however, the subglottic region is most commonly affected, representing approximately 64% of cases. ACC typically manifests as a slow-growing tumor with a pronounced tendency for perineural invasion and local recurrence. Current treatment strategies primarily involve surgical resection followed by adjuvant radiotherapy. Chemotherapy demonstrates limited efficacy and is generally reserved for advanced, recurrent, or metastatic disease. Given the rarity of this malignancy and the limited number of cases reported in the literature, we aim to contribute to the existing body of knowledge by presenting a clinical case of laryngeal ACC. Case Presentation: A 77-year-old male with a significant smoking history (more than 20 cigarettes per day for over 40 years) presented to our department in October 2023 with persistent dysphonia lasting several months. Endoscopic evaluation of the upper aerodigestive tract revealed an extensive neoplastic lesion involving the larynx. Contrast-enhanced computed tomography (CT) confirmed the presence and extent of the lesion. The patient subsequently underwent surgical resection and was referred for adjuvant postoperative radiotherapy. Unfortunately, the patient died of a myocardial infarction a few days before radiotherapy could be initiated. Conclusions: Due to the rarity of laryngeal adenoid cystic carcinoma, further studies are necessary to define optimal management strategies. Sharing clinical experiences and outcomes is essential, as there is currently no universally accepted treatment consensus for this uncommon malignancy. At the same time, our aim is to highlight the importance of histological subtype and perineural invasion which have to be considered as important prognostic factors when dealing with ACC.