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.