A 9 yr-old girl was admitted to Samsung Medical Center due to chronic productive cough and shortness of breath. During the infancy she developed cough and sputum, which was associated with frequent respiratory tract infection. Diarrhea persisted from 1 month of age regardless of the dietary formula and is unresponsive to antibiotics. Diarrhea led to poor weight gain and generalized edema, which necessitates hospitalization twice. Mantoux test performed at 10 months of her age because of chronic cough was positive (15x15 mm). Hence she was treated with anti-tuberculosis regimen for 9 months, but her symptoms did not improve. When she was 2 yr old, clubbing fingers were noticed, and at 5 yr of age, she received ventilator care due to severe respiratory distress. At that time, Pseudomonas aeruginosa was isolated from her sputum. Since then, her respiratory symptoms were waxed and waned, and she had been hospitalized several times.