Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of unknown etiology that occurs almost exclusively in women of reproductive age. High-resolution computed tomography (HRCT) reveals the striking feature of diffuse cystic changes throughout the lung parenchyma. The correct diagnosis may be delayed by several years after the onset of symptoms because of the rarity of the disease and the need for chest CT scans to identify the lung involvement. We describe a case of pulmonary LAM in a male patient associated with tuberous sclerosis complex (TSC), in whom the early stage of disease could be depicted by chest HRCT scans.