A rare case of incomplete tracheal duplication with severe unilateral lung hypoplasia is presented. Photo-documentation of the gross post-mortem specimens is presented so that the anatomical aspects of this unusual anomaly can be recognized and appreciated. Clinical information is presented in the hope that successful premorbid identification of potential complications of this anomaly could be made by future physicians. To our knowledge, this is the first reported case of pathologically-confirmed duplication of the trachea.