Immunoglobulin G4-related disease is a rare fibroinflammatory infiltrative condition with heterogeneous clinical manifestations. We present the case of a 64-year-old man that presented with hoarseness due to vocal cord involvement, a particularly rare manifestation. A large mediastinal mass compressing thoracic large vessels was also identified. The patient was initially treated with glucocorticoids but had relapses during glucocorticoid tapering. Rituximab was started after a careful pre-treatment evaluation of the infectious risk and treatment of comorbidities.