The authors report a case of a seventeen year old woman who was admitted to hospital for an extremely acute respiratory distress syndrome, which was revealed to be an exclusively pulmonary form of Goodpasture's syndrome. The positivity of the IgG immunofluorescence on open lung biopsy contrasted with the absence of circulating antibasement membrane antibody (ELISA). The combination of plasmaphoresis, steroid therapy and cyclophosphamide enabled a rapidly favourable outcome and the patient could be weaned of the support therapy.