Congenital complete and near-complete tracheal rings are extremely rare tracheal deformities. There are fewer than 100 cases of congenital complete tracheal rings, and only four cases of near-complete described in the literature. Long-segment occurrences of these anomalies are usually incompatible with life. We describe the presentation of the long-segment near complete tracheal ring deformity and long-segment anterior fusion of cartilage rings (cartilage sleeve deformity) in a living infant. The literature is reviewed, and the embryology, presentation, and management of these extremely rare anomalies are discussed.