Lymphangioleiomyomatosis (LAM) is a rare disease of unknown origin, that may be sporadic or develop in the course of tuberous sclerosis (TS). Patients do not present immune deficiency, but structural changes in the lung parenchyma (cysts) may encourage various infections, for example tuberculosis. Radiologic findings are often difficult to interpret, because of changes related to LAM itself. We present a young women with a history of TS and LAM, in whom protracting respiratory tract infection was finally diagnosed as tuberculosis. Initial diagnosis was based primarily on clinical signs and symptoms and treatment was started despite the negative result of the sputum microscopy for acid fast bacilli. In the course of treatment the diagnosis was supported by positive tuberculin skin test, interferon-gamma release assay and genetic test for M. tuberculosis in bronchoalveolar lavage fluid, and finally, positive sputum culture in liquid medium.