Shrinking lung syndrome is a rare manifestation of connective tissue diseases, namely systemic lupus erythematosus. It is characterised by reduced lung volumes and extra-pulmonary restrictive ventilatory pattern with good response to high-dose glucocorticoids alone or in combination with a second immunosuppressive agent. Here, we describe a case associated with mixed connective tissue disease and effectively treated with intravenous immunoglobulin.