Tracheal inflammatory pseudotumor (IPT) is a rare solid lesion with an unpredictable biological course. Treatment can vary and surgical resection may sometimes be necessary, even in pediatric age. We report the case of a 12-year-old male patient who presented to our institution with sudden dyspnoea after some months of wheezing and cough, wrongly considered and treated as asthma. Neck-chest CT-scan and fiberbronchoscopy showed an intraluminal tracheal mass, originating from the left antero-lateral wall at the level of the 5th cartilagineous tracheal ring, involving three rings, that was removed by rigid bronchoscopy. Histopathology revealed a tracheal IPT. Due to rapid tendency to recurrence of the lesion, two more endoscopic recanalizations were performed, but a new recurrence appeared, with CT evidence of transmural involvement of the tracheal wall. Resection of the three involved tracheal rings and termino-terminal tracheal anastomosis were successfully performed through cervicotomy and sternal split. CT-scan and fiberbronchoscopy at 17 months from surgery show a stable tracheal lumen without signs of recurrence. A tracheal IPT should be suspected in any pediatric patients with tracheal mass and asthmatic symptoms. After radical removal prognosis is generally excellent and recurrences after tracheal resection are rare.