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Abstract Pulmonary involvement of IgG4‐associated disease is a rare condition with no codified treatment apart from steroid administration. We report here the case of a patient with pulmonary involvement of IgG4‐RD successfully managed with Rituximab, in induction and maintenance therapy. This original case could support the use of Rituximab in rare situations of steroid‐resistant or steroid‐dependent pulmonary IgG4‐RD.