Laryngeal juvenile xanthogranuloma is rare and there were only four cases have been reported previously in English literature. We report a case of isolated JXG of larynx in the superglottic region. The mass was well-defined and homogeneous hypoechoic in ultrasonography while presented indistinct boundary and soft tissue density in Computed Tomography images. Radiological findings are nonspecific but could help to understand the extent of the lesion. We suggest that JXG should be considered as differential diagnosis for laryngeal stridor. Laryngeal ultrasound is safe and reliable, which could be considered a useful diagnostic tool for supplementing laryngoscopy.