The diagnostic criteria of definite LAM include characteristic or compatible features on high resolution CT of the chest and lung biopsy fitting the pathological criteria for LAM or characteristic high resolution computed tomography (HRCT)features and any of the following: renal angiomyolipoma, thoracic or abdominal chylous effusion, lymphangioleiomyoma or lymph node involvement by LAM, TSC or elevation of vascular endothelial growth factor-D (VEGF-D) in serum [2, 3]. Here, VEGFD is linked to lymphangiomyoma.