We report a case of pulmonary LAM occurring in a 24-year-old pregnant woman in whom large areas of air-space consolidation partly masked interstitial lung disease on plain radiography. For correct dignosis, high resolutionCT was considered to still be a potent method for the disclosure of typical interstitial lung changes, including thin-walled cysts. Follow-up CT showed progressive worsening of these interstitial lung lesions.