A case of congenital cystic bronchiectasis first becoming fully manifested at the age of 46 is described. The pathologic bronchi were limited to a single lobe of the lung and were associated with certain unusual congenital abnormalities including deafness, evaluation of the right diaphragm and high position of the cecum. The association of these disorders has not been described previously. The pathogenesis and diagnosis of congenital cystic bronchiectasis is discussed.