Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon lung disease that affects young adults aged 20 to 40 years with current or prior history of smoking. The pathologic cell type in PLCH is a dendritic cell of the monocyte-macrophage line that resembles cutaneous Langerhans cells. This report presents the case of a 42-year-old woman with PLCH. We discuss her clinical symptoms, diagnostic tests, and treatment plan, with a specific focus on the radiologic features. The patient exhibited a radiologic appearance similar to that of lymphangiomyomatosis with histologic evidence of PLCH.