From 1976 to 1989, 27 patients with pulmonary lymphangioleiomyomatosis were seen at our institution. Twenty-five had no signs or symptoms of tuberous sclerosis; 21 of these had abdominal imaging or surgical exploration to make the diagnosis of renal angiomyolipoma in seven patients, all female. Three presented with symptoms secondary to renal disease, three with pulmonary manifestations, and one with both. Renal disease was bilateral in four patients; four patients underwent nephrectomy and two tumors were embolized. All patients experienced spontaneous pneumothoraces during the course of their disease. There was one respiratory death. It is our belief that any patient presenting with angiomyolipoma and a history of pulmonary problems should be evaluated for pulmonary lymphangioleiomyomatosis with a limited computed tomography scan of the chest at the time of abdominal imaging. The surgical treatment of angiomyolipoma when associated with lymphangioleiomyomatosis is predicated on the ability to adequately manage the complicating lung disease perioperatively.