Abstract Objectives: Primary mucosa-associated lymphoma of the larynx is a very rare condition. The purpose of this presentation is to describe the clinical manifestations of this condition. We report 2 different clinical presentations of this case. Methods: Patient 1 was a 56-year-old patient presented with history of dysphagia and dysphonia. Laryngoscopy revealed an ulcerating mass in the left false cord and an ipsilateral vocal cord palsy. Histology revealed florid lymphocytic infiltration with scattered blasts. Immunohistochemistry was CD3+, CD20+, IgM+, and CD5-ve. Treatment with combination chemotherapy was instituted and the patient is alive and disease-free after 24 months. Patient 2 was a 64-year-old man with history of dysphonia. Laryngoscopy showed a submucosal spherical mass in the right false cord. Biopsy confirmed MALT lymphoma with the same immunohistochemistry. Treatment with chemotherapy was instituted and he is alive and well after 12 months. Results: We challenge the previously held view that MALT lymphomas of the larynx always have the same clinical presentation. The histological diagnosis of the condition consistent and is dependent on immunohistochemistry with positivity of the following markers: CD3, CD20, and IgM. The clinical picture can be varied and follow that of any malignancy in the larynx. Conclusion: Surgery only has a role in the diagnosis of this lympho-proliferative disease. Treatment should be directed to a multidisciplinary lymphoma team. The rarity of this tumor probably is due to the lack of recognition.