Juvenile xanthogranuloma (JXG) is an unusual histiocytosis by an unknown cause, which rarely occurs in the oral cavity. Its clinical presentation is non-specific, so prevents to differentiate it from other oral mucosal diseases. In addition, limited oral JXG cases have been reported so far, and clinical features and therapy of oral JXG are more and less different from cutaneous JXG. The purpose of this report is to present a case of JXG on the dorsal tongue and a review of relevant literature.

Juvenile xanthogranuloma (JXG) is a benign histiocytosis that occurs in the pediatric population. Cutaneous JXG is the most common form, while extracutaneous lesions, including oral JXG, is extremely rare. Cutaneous JXG can occur as multiple lesions and may have systemic visceral involvement, but this is not seen in oral JXG. In this case, we report a solitary oral JXG at the gingiva in a 3-year old male.