Lymphangioleiomyomatosis (LAM) is a progressive disease occuring exclusively in women of child-bearing age. It is a rare disease, possessing distinctive clinical, pathological and radiological findings. A case is reported of surgically- proved lymphangioleiomyomatosis in a 29-year-old woman with recurrent pneumothorax. Chest plain film showed bilateral reticular and honeycomb patterns with a normal lung volume. High resolution computed tomography (HRCT) revealed characteristic diffuse cystic changes of the lungs. Recognition of this characteristic radiological feature is helpful in suggesting the diagnosis of LAM.