Coexisting lymphangioleiomyomatosis and bilateral angiomyolipomas in a patient with tuberous sclerosis.

A patient with tuberous sclerosis and known bilateral angiomyolipomas presented with a complete pneumothorax prior to scheduled renal surgery. Evaluation revealed the presence of pulmonary lymphangioleiomyoma, an unusual entity known to coexist with tuberous sclerosis. Pulmonary decortication and conservative renal surgery resulted in a satisfactory long-term result.