Mantle cell lymphoma is a variant of non-Hodgkin's lymphoma and is known as a mature peripheral B-cell lymphoid neoplasm. Lymphomas of the larynx and nasopharynx are rare; mantle cell lymphoma is exceedingly rare. In most mantle cell lymphoma cases, the tumoral lymphoid cells express nuclear cyclin D1 due to a t(11;14) chromosomal translocation between an IGH gene and CCND1. This is considered to be an important genetic event in mantle cell lymphoma. There are case reports in the literature on various histologic types of non-Hodgkin's lymphomas which affect the nasopharynx and larynx simultaneously. However, to the best of our knowledge, mantle cell lymphoma involvement of the nasopharynx and larynx simultaneously has not been reported. In this case report, the detailed clinical manifestations and pathological findings of a mantle cell lymphoma patient have been presented along with a literature review of mantle cell lymphoma.