Primary tracheal neoplasms are extremely rare lesions in the pediatric age group. This study reviews the English-language literature to better characterize these lesions in children and reports 2 additional patients. Reports of only 36 infants and children through adolescence with primary tracheal neoplasms were discovered after an exhaustive literature review of the last 30 years. The data are analyzed with regard to pathology, demographics, symptomatology, site, and percent luminal obstruction. We report 2 additional patients with photographic documentation, imaging studies, and histopathology. Of the 36 previously reported lesions, 64% were characterized as benign and 36% as malignant. Fifty-six percent of all lesions were initially misdiagnosed as asthma. The most common site was the posterior membranous wall of the cervical trachea. In 14 (39%) of the 36 patients, the lesions obstructed more than 50% of the lumen at the time of diagnosis. The timely diagnosis of tracheal masses depends upon maintaining a high index of suspicion and conducting an efficient workup, including definitive evaluation by bronchoscopy. The evaluation and the differential diagnosis of tracheal neoplasms in the pediatric population is discussed.