JXG is a benign idiopathic cutaneous granulomatous tumor and the most common form of non-Langerhans cell histiocytosis (non-LHC).

They may also appear at the groin, scrotum, penis, clitoris, toenail, palms, soles, lips, lungs, bone, heart, and gastrointestinal tract more rarely.

[4] JXG usually manifests with multiple lesions on the head and neck in cases with children under six months of age.

Treatments that have been used include surgical excision, intralesional steroid injection, cryotherapy, and low dose radiotherapy.

Juvenile variety xanthogranuloma can be distinguished from xanthoma by the spread of the lesion and the lack of lipid abnormalities.