Juvenile xanthogranuloma is a benign non-Langerhans cell histiocytosis histogenetically associated with dermal macrophages that more often appears as papules or yellow-red nodules in the skin or soft tissues. The disease mainly affects people during the first years of life with a mean age of 4.5 years old at diagnosis; however, many cases affecting adults have also been described. It may develop synchronically or metacronically with other histiocytosis, and may also evolve from a previous histiocytic condition like Langerhans cell histiocytosis. The oral mucosa can also be affected, more often the cheek-mucosa, tongue and gingiva, commonly presenting as painless submucosal nodules of normal-colored to yellowish to brownish aspect. These lesions are usually small, soft and non-ulcerated. Microscopically, it presents as an unencapsulated, circumscribed, widespread proliferation of foamy or eosinophilic histiocytic cells, and the presence of multinucleated giant cells consistent with Touton cells is considered a typical histologic finding. Juvenile xanthogranuloma is a self-limited condition, and some cutaneous lesions may undergo spontaneous regression. On the other hand, systemic juvenile xanthogranuloma may have a more aggressive clinical course. Oral lesions usually do not regress spontaneously, and they are more often treated by conservative surgical removal.