We experienced one case of pulmonary lymphangioleiomyomatosis in 26-year-old female patient. She had taken antituberculous medication under the impression of miliary tuberculosis on simple chest X-ray at peripartum period. On outpatient follow-up she complained of progressive exertional dyspnea in spite of medication. Through careful history taking and physical examination, high resolutional CT, and open lung biopsy she was diagnosed as pulmonary lymphangioleiomyomatosis combined with incomplete type of tuberous sclerosis. So, we presented the case with the brief review the literatures.