Lymphangioleiomyomatosis (LAM) is a form of cystic lung disease, quite rare disease of unknown cause that traditionally affects young women of reproductive age. It is characterized by excessive proliferation of immature smooth muscle and spindle cells (LAM cell) along the axial lymphatics, leading to progressive cystic lung destruction, lymphatic obstruction, and abdominal tumours. LAM is almost universally fatal without a lung transplant. Because of its rareness and nonspecific presenting symptoms, patients often receive a missed or delayed diagnosis. We present the case of a 68-year-old postmenopausal woman from maldives who presented with fever, cough with mucopulent phlegm and worsening orthopnoea was ultimately determined to be due to LAM with Acenetobactor infection. LAM may be more commonly recognised than previously and is imperative for primary care providers to be able to recognize this disease so they can make prompt referrals to appropriate specialty centers.
