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Lymphoepithelial carcinoma (LEC) of the larynx is a rare tumor comprising 0.2% of all laryngeal neoplasms. To our knowledge, less than 50 cases of LEC of the larynx are reported in the literature. The lack of specific guidelines leads to uncertainty on this rare entity’s diagnosis, treatment, and follow-up. The association of Epstein–Barr virus as a predisposing factor of LEC has been proposed in a few reports. Although the occurrence of LEC in the larynx is infrequent, it is essential to differentiate it from squamous cell carcinoma. Immunohistochemistry should be performed to demonstrate cytokeratin expression. It is a very aggressive tumor with high metastatic potential; however, it is hard to comment on the disease outcome of LEC of the larynx because only case reports are available in the literature. Here, we report a case of LEC of the larynx (CK +ve, CD 45 –ve, and EBER-ISH –ve) in a 49-year-old male. Initially, the patient was planned for surgery but defaulted. He reported back after 4 months, and there was clinicoradiological disease progression. Because of the unresectable status and poor general condition, he received palliative radiotherapy only. Additionally, we have conducted a pooled analysis of the individual cases of LEC of larynx available in the literature to understand the clinical behavior to optimize the treatment of these patients.