Postoperative pneumothorax after nephrectomy in a patient with tuberous sclerosis and undiagnosed lymphangioleiomyomatosis

• Before surgery in a patient with known tuberous sclerosis (TS) and particularly of genetic origin in a young woman, lymphangioleiomyomatosis (LAM) should be evoked. • Its diagnosis should be ensured before proceeding to surgery to avoid the risk of respiratory complications including pneumothorax . • In patients with a known combination of LAM and TS, anesthetic and surgical management is aimed to reduce both LAM-associated risk (i.e. respiratory complications) and TS-associated risk (neurological, cardiac and renal involvement).