Immotile cilia syndrome is a congenital structural abnormality of cilia. The structural abnormality is lack of dynein arm or defective radial spoke or microtubular transposition. In this syndrome, ciliary movement is completely absent or dyskinetic and half of this syndrome shows Kartagener`s triad. We report a 13-year-old girl who had immotile cilia syndrome with Kartagener`s triad. She had been suffering from frequent respiratory infection, hemoptysis, large amount of sputum, and sinusitis. Bronchography revealed tubular bronchiectasis in right lower lobe and that lobe was resected for treatment of bronchiectasis. Histological examination of resected bronchus showed chronic bronchiectasis and electronmicroscopically complete lack of both inner and outer dynein arms. Hospital course was uneventful and symptoms were much improved.