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A 49-year-old woman with a history of generalized lymphangiomyomatosis or lymphangioleiomyomatosis (LAM), including angiomyolipomas of the liver and kidney and known LAM of the lung, presented with symptomatic cholelithiasis. She underwent an uneventful laparoscopic cholecystectomy and was discharged home the following day. Careful patient selection, preoperative stratification, and optimization of pulmonary status are all necessary prior to electing to operate on patients with known LAM. A CT scan of the abdomen is of value to delineate the anatomy of the angiomyolipomas and their proximity to the operative site. These preliminary precautions, combined with careful surgical technique, are essential for a successful outcome with minimal morbidity.