Juvenile Xanthogranuloma (JXG) is a dendritic cell related histiocytic disorder which usually presents in the first year of life as a solitary cutaneous granuloma. Isolated presentation in the upper airway is very rare but can result in severe respiratory distress, especially in young children. We present the case of a 5-month-old male with an isolated subglottic JXG lesion. Endoscopic excision provided symptomatic relief and avoided the need for tracheostomy. The lesion has completely resolved 17 months later. Surgical excision without tracheostomy was the treatment of choice in two of the four additional cases of upper airway JXG presented in the literature. JXG has an excellent prognosis with spontaneous regression over time. Histology alone is frequently inadequate to differentiate JXG from the more common Langerhans Cell Histiocytosis (LCH), which carries a much less favorable prognosis. The evolving field of immunohistochemistry provides an essential tool to establish the correct diagnosis. The typical phenotype of JXG is Factor XIIIa+/Fascin+/CD68+/CD163+/CD14+/CD1a-/S100-.