Endobronchial tumors (ET) are unusual and mostly malignant, presenting with non-specific symptoms that often delay appropriate diagnosis and treatment. Lymphomas in the airway represent less than 1% of pulmonary malignancies and require multidisciplinary approach for their management. We present a case of a 48-year-old male former smoker with a one-year history of recurrent respiratory infections and new-onset shortness of breath. Diagnostic tests included a chest computed tomography (CT) reporting the presence of an endobronchial mass and neck and cervical lymph node biopsies with inconclusive results. Bronchoscopy was successfully performed for tumor resection, improving the patient's respiratory symptoms. Histological analysis described an extranodal marginal zone B-cell lymphoma (ENMZL) with plasmacytic differentiation; a subtype of non-Hodgkin's lymphoma (NHL) in mucosa-associated lymphoid tissue (MALT), rarely found as an endobronchial growth. ET should be considered in the setting of persistent and worsening respiratory symptoms. ENMZL with plasmacytic differentiation is rarely found as an ET and diagnosis requires bronchoscopic intervention and extensive immunohistochemical analysis.