Tracheal hamartoma represents an oddity in children. Only one case was found in the English literature as a distal tracheal intraluminal lesion causing obstructive symptoms. All other reported cases were in order patients, who were presumed to have asthma or chronic obstructive pulmonary disease. The authors report on a 21-month-old girl who presented with an anterior neck mass fixed to the trachea. Imaging studies showed a lesion (2.5 x 2.3 x 1.7 cm) anterior to the right lobe of the thyroid, extending to the trachea posteriorly and down to the thoracic inlet inferiorly. The mass had a mixture of soft tissue densities with a focus of calcification. Results of thyroid studies were normal, and there was no adenopathy. Neck exploration showed a white, firm, lobulated mass fixed to the trachea, which was resected completely. The postoperative course was unremarkable. Histological studies showed a mixture of mature cartilage, fat, fibroconnective tissue, and spindle cells with myxoid degeneration, consistent with tracheal hamartoma. Based on a literature search, this is the first reported case of extraluminal tracheal hamartoma presenting as a neck mass in a pediatric patient. Copyright (C) 1996 by W.B. Saunders Company