Despite the absence of other supportive clinical and diagnostic features of LAM such as renal AMLs, neurocutaneous features of tuberous sclerosis, and chylous pleural effusions, a provisional diagnosis of LAM was made based on the patient demographics and chest CT findings. A VAT pleurodesis and lung biopsy was performed but histology was inconclusive for LAM. The case was discussed at a tertiary hospital interstitial lung disease multidisciplinary team meeting and the diagnosis of sporadic LAM was supported. The patient was referred to a lung transplant center.