Lymphoepithelial carcinoma of the larynx is a rare tumor, as this histological entity is mostly diagnosed in nasopharynx. However, it may be present in other non-nasopharyngeal sites and it is extremely rare in the larynx. The authors present a case of a 59-year-old man who presented to the Otorhinolaryngology-Head and Neck Surgery Department complaining of a long-standing dysphonia, odynophagia, and dysphagia. The clinical examination revealed a laryngeal tumor involving the right epiglottis, right aryepiglottic fold, and ipsilateral false vocal fold. It presented with ispilateral neck lymph node extension. Multiple biopsies of the laryngeal lesion were performed under local anesthesia and the histological examination showed a poorly differentiated squamous cell carcinoma. After discussing the case in a multidisciplinary tumor board, a total laryngectomy with a bilateral neck dissection was performed and the histological specimen showed a lymphoepithelial carcinoma. Although immunostaining with LMP-1 antibody was negative, in situ hybridization for Epstein-Barr virus was positive. He underwent adjuvant chemoradiation. He is now at 9-months follow-up period, with no evidence of disease. Lymphoepithelial carcinoma of the larynx is an extremely rare disease, with an aggressive pattern. Epstein-Barr virus-associated lymphoepithelial carcinoma has been exceptionally reported. A correct diagnosis and close collaboration with pathologist is crucial to achieve the best treatment strategy. We present this case to discuss the clinical and histology findings and the different therapeutic aspects of this uncommon histological subtype carcinoma.