Lymphangioleiomyomatosis, a rare disease occurring in women of childbearing age, is characterized by proliferation of smooth muscle in pulmonary lymphatic channels and mediastinal and abdominal lymph nodes. Chest radiographs typically reveal interstitial disease with normal lung volume. Pneumothorax and pleural effusions may be present. CT scans in eight patients with biopsy-proved pulmonary lymphangioleiomyomatosis were reviewed. The prominent feature of the disease was multiple thin-walled cysts throughout the lungs, best visualized on scans made with 1.5-mm collimation. Mediastinal and/or retrocrural lymphadenopathy, often not appreciated on the chest radiograph, was present in four of eight patients. CT can suggest a diagnosis of lymphangioleiomyomatosis when diagnosis by clinical presentation and chest radiographs is uncertain.