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A case history of ataxia-telangiectasia has been presented. A corrosion model was prepared from the left lung and was dissected and studied under the stereomicroscope. Saccular bronchiectasis was found in many bronchi and bronchioles. However, those terminal bronchioles and structures peripheral to it which were derived from bronchiectatic bronchi showed constrictions rather than dilatations. Alveoli in such areas appeared deformed, but apparently still functioning since normal capillary networks could often be demonstrated. Constrictions at the point of branching of small peripheral pulmonary artery branches were frequently seen. Serial histologic sections revealed the presence of cushions at the points of constriction. Another vascular abnormality consisted of numerous anastomoses between smaller branches of the pulmonary artery forming networks of seemingly useless loops. In some areas, the capillaries appeared dilated, were club-shaped and had aneurysm-like dilatations.