The authors describe a patient with histologically confirmed pulmonary lymphangiomyomatosis and thin-section computed tomography findings mimicking Langerhans cell histioctytosis. The description emphasizes the nonspecificity of the computed tomography findings in this patient. This report also suggests that the computed tomography diagnosis of lymphangioleiomyomatosis can be difficult at an early stage of the disease and should be complemented by biopsy verification.