Pulmonary 1ymphangioleiomyomatosis is a very rare disease mainly arising in reproductive-aged women. Pulmonary 1yjphangioleiomyomatosis as a pulmonary involvement of tuberous sclerosis is found in only 1 out of 100 patients. Pulmonary involvement in pulmonary 1ymphangioleiomyomatosis itself and that as a pulmonary manifestatio of tuberous sclerosis has been considered very similar with regard to clinical, radiologic, and pathologic manifestations. We report 1 case of pulmonary 1ymphangioleiomyomatosis as a pulmonary manifestation of tuberous sclerosis in a 39-year-old Korean woman.