Bronchomalacia has not previously been described in association with Larsen syndrome. In this article, three children with Larsen syndrome are reported. All three patients have tracheomalacia, and one of them has diffuse bronchomalacia diagnosed at flexible fiberoptic bronchoscopy. The patients' symptoms of airway obstruction include stridor, cyanosis, apnea, and recurrent pneumonias. Recognition of these abnormalities may allow the institution of supportive therapy.