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Abstract We report a 35‐year‐old woman who presented with dyspnoea and chest pain for 1 week. High‐resolution computed tomography (HRCT) thorax revealed bilateral pneumothoraces with diffuse lung cysts. Bilateral intercostal chest tubes were inserted, and there was a persistent air leak (PAL) bilaterally. We performed an autologous blood patch pleurodesis (ABPP) for the left PAL. For the right PAL, she underwent a successful right video‐assisted thoracic (VATS) surgery, wedge biopsy, and surgical pleurodesis. Histopathology examination confirmed the diagnosis of lymphangioleiomyomatosis (LAM). The left pneumothorax recurred. An indwelling pleural catheter (Rocket® IPC™; Rocket Medical plc; WASHINGTON) was inserted and the patient was discharged after 1 day with an atrium pneumostat (Pneumostat™; Atrium Medical Corporation, Hudson, NH, USA) chest drain valve. The patient was initiated on Sirolimus 2 mg daily. The left PAL resolved at 6 weeks. This case highlights the benefit of IPC with an ambulatory pneumothorax device in a patient with LAM with PAL.