Extranodal laryngeal lymphoma is extremely rare. We report a case of primary laryngeal lymphoma in a 76-year-old man who had presented with a 7-week history of progressive hoarseness. Laryngoscopy revealed asymmetry of the right false vocal fold. Pathology of a deep biopsy specimen identified a malignant, diffuse, CD20-positive, B-cell lymphoma. The stage 1E lymphoma completely resolved after treatment with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) and rituximab. Despite its relative rarity, the consequences of a missed diagnosis warrant vigilance for this type of laryngeal tumor.