Airway manifestations of inflammatory bowel disease are rare in pediatrics. This case describes a nine-year-old female with ulcerative colitis (UC) with progressive stridor and dyspnea for two months. Severe upper airway obstruction was noted on spirometry. CatScan (CT) scan of the neck and chest revealed tracheal narrowing with circumferential, heterogeneous soft tissue thickening and posterior wall nodularity. Bronchoscopy visualized granulation tissue of the large airways and an ulcerative lesion to the right mainstem. Consultation and evaluation by gastroenterology, oncology, and rheumatology determined a diagnosis of extraintestinal manifestations of UC. Systemic steroids led to symptom resolution and improvement in lung function.