Pulmonary alveolar microlithiasis is a rare genetic disorder, inherited autosomally recessively, which is characterized by intra-alveolar deposition of microliths built mostly of calcium salts and phosphorus. This case study describing management of patient with pulmonary alveolar microlithiasis. A 49-year-old woman, diagnosed with pulmonary microlithiasis in 1979 was admitted to Pneumology Department due to increased dyspnea. On admission there were no clinical signs of active infection. The chest computer tomography scan confirmed the presence of advanced microlithiasis. Pulmonary function test revealed mild restriction with moderate diffusion impairment, due to severe hypoxemia present on 6-minute walking test patient was sent for specific assessment to local lung transplant team in Zabrze for consideration for lung transplantation. According to International Society for Heart & Lung Transplantation guidelines the patient was observed in 6 months intervals to reveal whether further disease progression will be observed. Clinical condition of our patient does not correlate with radiological scans, severe respiratory symptoms and cardiological complications. Computer tomography scan should not be the only indication for lung transplant.