Lymphangioleiomyomatosis is a rare and complicated disorder that affects the young, almost exclusively women. It may be associated with the tuberous sclerosis complex, which includes renal angiolipoma, chylothorax and lymph node myomatosis. Its clinical pulmonary manifestations vary from simple cough to the development of recurrent pneumothoraces, hemoptysis, and even complicated pleural effusions. Progressive dyspnea develops as the disease evolves. Eventually most patients require lung transplantation. This wide array of symptoms and signs makes the differential diagnosis extensive, and the clinician must be familiar with this disorder to arrive promptly to the correct diagnosis. We report a case of a 36-year-old woman with a long history of recurrent pleuritic chest pain with associated dyspnea before being diagnosed with lymphangioleiomyomatosis. A review of the literature pertinent to this case is provided.